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Transcript
SHORT NOTES ON
CLINICAL NEUROLOGY
THIRD EDITION
Abdel-Latif Moussa Osman
M.D., D.P.M. & N., D.M.
Professor & Chairman
Departments of Neurology
Faculty of Medicine
Al-Azhar University
CAIRO
2004
1
‫بسم هللا الرحمن الرحيم‬
‫سا َب ْ‬
‫س َب ْ‬
‫ت‪،‬‬
‫"الَ ي ِّ‬
‫ُكل ُ‬
‫ت َو َ‬
‫علَيْهاا َ َماا ا ْكَ َ‬
‫ف هللا نَ ْفسا ِّإالَّ ُو ْس َع َها‪ ،‬لَ َها َما َك َ‬
‫اارا‬
‫علَيْناا َ ِّإ ْ‬
‫َربَّنَا الَ ت ُ َؤاخ ْذنَا إن نَسينَا أ َ ْو أ َ ْخطأْنَا‪َ ،‬ربَّناا َ َوالَ ت َ ْْماع َ‬
‫ان َْبْلنَاا‪َ ،‬ربَّناا َ َوالَ ت ُ َْ ِّم ْلناا َ َماا الَ َ‬
‫لاْا َ لَناا َ‬
‫علَا الاذينَ ِّم ْ‬
‫َكما َ َح َم ْلََهُ َ‬
‫علَا‬
‫ار َح ْمنااَ‪ ،‬أ َ ْن َ‬
‫فار لَناا َ َو ْ‬
‫عنَّاا َوا ْْ ْ‬
‫به‪ ،‬واع ُ‬
‫ماالَناا َ رَا ْن ْارنَا َ‬
‫ات ْ‬
‫ْف َ‬
‫ْالقَ ْا ِّم ْال َكارِّ ِّرين"‬
‫(البقرة ‪)286‬‬
‫"‪ ...‬رأ َ َّمااا َّ‬
‫ََ ًُفَااام‪َ ،‬وأ َ َّمااا َمااا يَنفَ ا ُك النَّااا َ ريم ُك ا ُ رِّ ا‬
‫الببَ اَ ُ رَيَااذُ ُ‬
‫ض‪"... ،‬‬
‫ْ‬
‫األر ِّ‬
‫(الرعَ ‪)17‬‬
‫‪Short Notes on Clinical Neurology, 3rd ed. 2004‬‬
‫‪All rights reserved. No part of this book may be reproduced in any form‬‬
‫‪or by any means, without written permission of the author.‬‬
‫‪ANDALOSIA FOR PRINTING‬‬
‫‪TEL: 4024587‬‬
‫‪National Deposit No‬‬
‫‪ISBN:‬‬
‫‪2‬‬
To The Memory of
My Father & Mother
3
4
Preface to the Third Edition
The previous two editions of this booklet have been very useful for precisely
revising a lot of knowledge in a short time. It has been of great help to
medical students, general practitioners, neurophychiatrists & neurosurgeons.
The overall contents have been thoroughly revised & new interesting topics
were added.
I hope this new edition will be more informative & interesting for my
students & colleagues.
Looking forward to hearing from you.
Abdel-Latif Moussa Osman, MD
14 Shehab Street,
Mohandseen, Giza, Egypt
Telephone & Fax: (202) 7605566
(202) 7613513
Mobile
: 0123797586
E-mail: aaosman@hotmail.com
Cairo, 2004
5
Preface to the First Edition
This booklet is intended as a guide for medical students, general practitioners
& residents in internal medicine, neurology & neurosurgery. It summarizes
the criteria of diagnosis of common neurological diseases & gives a brief
account of some important clinical data & diagnostic studies. This guide is
not a substitute for textbooks of neurology, but it can help those who have
studied the texts to crystallize their knowledge.
I am greatly indebted to my wife & children for their love, devotion, patience
& understanding.
I am also indebted to many of my professors, friends, colleagues, students &
patients for their cooperation & help.
Abdel-Latif Moussa Osman,
MD January, 1985
6
CONTENTS
NEUROLOGY SHEET
INVESTIGATIONS
NEUROLOGICAL SYMPTOMATOLOGY
 Weakness
 Wasting
 Hypotonia
 Hypertonia
 Involuntary Movements
 Parkinson's Disease
 Chorea
 Athetosis
 Dystonia
 Tremors
 Sensory Impairment
 Sensory Ataxia
 Gait Disorders
 Neurogenic Bladder
 Raised Intracranial Pressure
 The Facial Nerve
 Facial Paralysis
 Bell’s Palsy
 Facial Pain
 Ptosis
 Nystagmus
 Pseudobulbar Palsy
 Progressive Bulbar Palsy
 Hemiplegia
 Paraplegia
 Cauda, Conus & Epiconus Lesions
DISORDERS OF CONSCIOUSNESS & EQUILIBRIUM
 Coma
 Syncope
 Vertigo
 Narcolepsy
 Autonomic Dysfunction
7
PAINFUL SYNDROMES
 Migraine & Cluster Headache
 Tension-Type Headache
 Headache Presenting in Emergency Department
 Trigeminal Neuralgia
 Low Back Pain & Sciatica
 Cervical Spondylosis & Brachialgia
SEIZURE DISORDERS
 The Epilepsies
 Paroxysmal Conditions Resembling Epilepsy
 Drug Treatment of Epilepsies (Antiepileptic Drugs)
 Tonic-Clonic Status Epilepticus
CEREBROVASCULAR DISEASES
 Blood Supply of the Brain
 Syndromes of the Cerebral Arteries
 Cerebral Embolism
 Cerebral Thrombosis
 Cerebral Haemorrhage
 Cerebral Arteriosclerosis
 Subarachnoid Haemorrhage
 Transient Ischemic Attacks (TIAs)
 Reversible Ischemic Neurologic Deficit (RIND)
 Acute Strokes
 Hypertensive Encephalopathy
 Subarachnoid Hemorrhage (SAH)
 Intracerebral Hemorrhage
 Vasculitis of the CNS
CNS INFECTIONS
 Acute Meningitis
 Tuberculous Meningitis
 Viral Infections of the CNS
 Herpes Simple & Virus Encephalitis
 Herpes Zoster
MOVEMENT DISORDERS
 Parkinson’s Disease (PD)
 Huntington’s Disease (HD)
 Rheumatic (Sydenham’s) Chorea
8
 Tremor
 Cerebellar Ataxia
PERIPHERAL NERVE DISEASES
 Polyneuropathies
 Guillain-Barre` Syndrome (GBS)
 Non-inflammatory Peripheral Neuropathies
NEUROMUSCULAR JUNCTION DISEASES
 Myasthenia Gravis (MG)
 Myasthenic & Cholinergic Crisis
 Congenital Myasthenic Syndromes
 Neonatal Myasthenia
 Lambert-Eaton Myasthenic Syndrome (LEMS)
MUSCULAR DISEASES
 Muscular dystrophies
 Duchenne Muscular dystrophy
 Limb-Girdle Muscular dystrophy
 Facioscapulohumeral Muscular dystrophy
 Myotonias
 Myotonic Dystrophy
 Myotonia Congenita
 Periodic Paralysis
 Polymyositis
DEMYELINATING DISEASES
 Multiple Sclerosis (MS)
 Optic Neuritis
 Neuromyelitis Optica
 Acute DEM
 Acute Transverse Myelitis
NUTRITIONAL DISEASES
 Pellagra
 Beriberi
 Subacute combined Degeneration of the spinal Cord
NEURODEGENERATIVE DISEASES
 Alzheimer’s Disease (AD)
 Amyotrophic Lateral Sclerosis (ALS)
 Normal Pressure Hydrocephalus (NPH)
CNS NEOPLASMS
9
 Brain Tumours
 Primary Intracranial Tumors
 Childhood Brain Tumors
 Spinal Cord compression
 Carcinomatous Meningitis
 Pseudotumor Cerebri
NEUROLOGICAL COMPLICATIONS OF D. MELLITUS
PSYCHOTROPIC DRUGS
 Anxiolytics (Minor Tranquilizers)
 Neuroleptics (Major Tranquilizers)
 Antidepressants
 Hallucinogenics (Psychodysleptics)
SEDATIVES & HYPNOTICS
SKELETAL MUSCLE RELAXANTS
NARCOTICS ANALGESICS & ANTAGONISTS
10
NEUROLOGY SHEET
HISTORY
I. Personal History :
* Name : ... ... ... ...
* Age: ... ... ...
* Sex : ... ...
* Occupation : ... ... ...
* Address : ... ...
* Habits :
* Marital state : ... ... ...
* Handedness : ... ... ... ... ...
II. Complaint:
1. ... ... ... ... ... ... ... ... ... ...
2. ... ... ... ... … ... ... ... ... ...
3. ... ... ... ... … ... ... ... ... ...
III. Family History :
1. Positive blood consanguinity between the parents.
2. Similar condition in the family.
IV. Past History : (Trauma, fever ,Syphilis, T.B., diabetes, otitis media,
rheumatism, billharziasis,.. .etc)
V. History of the present illness :
(1) Duration: ... ...
(2) Onset: ... ...
(3) Course: ......
(4) Symptomatology (Chronologically): ... ... ... ... ... ... ...
(5) Inquiry about other important symptoms (Symptoms of increased ICT,
Cranial nerve affection, Motor, Sensory or Sphincteric troubles).
EXAMINATION
I. The Mental State :
(1) Attention: ... ... ...
(2) Orientation (for Time, Space & Persons)
(3) Memory (for Recent & Remote events) ... ... ...
(4) Mood:... ... ... ...
(5) Delusions & Hallucinations.
II. Speech & Artlciglatfosi: ... ... ... ... ... ... ... ... ... ...
III. Cranial Nerves.
Rt.
Lt.
- Olfactory: ... ... ... ... ... ... ... ... ... ... ... ...
- Optic (2nd):
* Visual Acuity: ... ... ... ... ... ... ... ...
: ... ... ... ... ... ...
* Fundus: ... ... ... ... ... ... ... ...
- The 3rd, 4th & 6th:
... ... ... ... ... ... ... ... .. ...
11
* Pupil. ... ... ... ... ... ... .., ... .. . .
* External Ocular Movements (Individual & Conjugate)
* Nystagmus: ... ...
- Trigeminal:
* Motor (temporal muscles, masseters & pterygmds)
* Sensory: ... ... ... ... ... ... ... ...
* Corneal Reflex ... ... ... ... ... ...
* Jaw jerk ... ... ... ... ... ... ... ...
- Facial:
* Motor
 Upper face ... ... ... ... ... ... ...
 Lower face ... ... ... ... ... ...
* Taste ... ... ... ... ... ... ... ...
- Auditory (8th):
* Cochlear
 Whispered Voice ... ... ... ... ... ... ...
 Watch test ... ... ... ... ... ... ... ...
 Rinne's test ... ... ... ... ... ... ... ...
 Weber's test ... ... ... ... ... ... ... ...
* Vestibular
 Rotational test ... ... ... ... ... ... ... ...

Caloric test .. ... ... ... ... ... ... ... ...
- Glossopharyngeal & Vagus (9th & 10th):
* Movement of the Palate & Pharynx (on saying Ah)
* Palatal & Pharyngeal reflexes (gag reflex) ...
* Vocal Cords .. ... ... ... ... ... ... ... ... ...
- Accessory (11 th):
* Trapezii .. ... ... ... ... ... ... ... ... ... ...
* Sternomastoids ... ... ... ... ... ... ... ...
- Hypoglossal (12th): Tongue ... ... ... ... ... ... ... ... ...
IV. The Motor System :
1. Muscle Status & Deformities: ... ... ... ... ...
2. Muscle Tone: ... ... ... ... ... ... ... ... ...
3. Muscle Power: ... ... ... ... ... ... ... ... ... ...
4. Involuntary Movements (including fasciculation)
V. The Sensory System :
1. Superficial Sensations:
* Pain ... ... ... ... ... ... ... ... ... ...
* Temperature ... ... ... ... ... ... ... ...... ...
12
* Touch ... ... ... ... ... ... ... ... ... ...
2. Deep Sensations:
* Position Sense ... ... ... ... ... ... ...
* Sense of Movement ... ... ... ... ... ... ... ...
* Vibration Sense ... ... ... ... ... ... ...
3. Cortical Sensations:
* Stereognosis ... ... ... ... ... ... ... ...
* Graphoesthesia ... ... ... ... ... ... ... ...
* Two point Discrimination ... ... ...... ... ...
* Sensory Inattention ... ... ... ... ... ... ...
VI. The Reflexes :
U.L.:
* Biceps Jerk ... ... ... ... ... ...
* Supinator Jerk ... ... ... ... ...
0==Absent
* Triceps jerk ... ... ... ... ... ...
±==diminished
* Finger jerks ... ... ... ... ... ...
+==normal
++ to ++++
Abdominal Reflexes: ... ... ... ...
= exaggerated
L.L.
* Knee Jerk ... ... ... ... ... ... ... ...
* Ankle Jerk ... ... ... ... ... ... ... ...
* Clonus (ankle or patellar) : ... ...
Plantar Reflexes:
 ===flexor,  ==extensor
VII. Co-ordination:
1. Finger-to-nose test ... ...
2. Finger-to-finger test ...
3. Heel-knee test ... ... ...
VIII. Gait: ... ... ... ... ... ... ... ...
IX. Skull & Spine: ... ... ... ...
X. Signs of Meningeal irritation :
1. NeckStiffness ... ... ... ... ... ...
2. Kernig's sign ... ... ... ... ... ......
3. Straight Leg Raising (Lasegue's sign) ... … …
XI. Palpation & Auscultation of Carotids: … …
XII. General Examination :
1. Chest: ... ... ... ... ... ... ...
2. Heart: ... ... ... ... ... ... ...
3. Abdomen: ... ... ... ... ...
4. Glands: ... ... ... ... ... ...
13
5. Pulse: . ... ... ... ... ... ...
6. Temperature: ... ... ... ...
7. B.P.: ... ... ... ... ... ... ...
PROBABLE DIAGNOSIS & DIFFERENTIAL DIAGNOSIS:
1. Clinical Diagnosis. ... ... ... ... ... ...
2. Etiological Diagnosis. ... ... ... ... ... ...
3. Anatomical Diagnosis. ... ... ... ... ... ...
4. Pathological Diagnosis. ... ... ... ... ... ...
INVESTIGATIONS:
* CSF: ... ... ... ... ... ... ...
* Radiography: ... ... ... ...
* Blood:... ... ... ... ... ... ...
* EEG:... ... ... ... ... ... ...
* Urine:... ... ... ... ... ... ...
* EMG: ... ... ... ... ... ...
* TCD:
* Stools: ... … ... ... ... ...
* Other Investigations: ... ...
* Evoked potentials: ... ... ...
* VCPs: ... ... ... ... ... ...
* BAEPs: ... ... ... ... ... ...
* SSEPs: ... ... ... ... ... ...
14
INVESTIGATIONS
I- THE CEREBRO-SPINAL FLUID (CSF):
1. Lumbar Puncture:
Indications:
(a) Diagnostic :
i. To obtain CSF for analysis.
ii. To diagnose subarachnoid haemorrhage.
iii. To measure CSF pressure.
iv. To introduce myodil for myelography.
v. To introduce air for air encephalography.
(b) Therapeutic:
i. To introduc antibiotics e.g. in meningitis.
ii. Spinal anaesthesia.
iii. Alcohol injection to relieve pain.
iv. Removal of toxic & inflammatory substances & reduction of CSF
pressure in meningitis, encephalitis & cerebral haemorrhage.
Contraindications:
i. Local sepsis e.g. bed sores, for fear of meningitis.
ii. Increased ICT with papilloedema, for fear of medullary conisation.
iii. Posterior fossa tumours with incipient brain stem compression.
iv. Marked hypotension, advanced chest or heart disease & bad general
condition of the patient.
Complications:
(a) Immediate:
* Neurogenic shock. Hypotension. Medullary conisation. Broken
needle.
* Accidental injection into a vein leading to shock. Sensitivity to the
injected drug.
(b) Delayed :
i. Headache: due to leakage of CSF through the puncture hole.
Treated by : Plenty fluids, ephedrine, pituitrin & lying flat.
ii. Infection leading to meningitis.
iii. Neurological complications e.g. 6th nerve palsy, sciatica or cauda
equina lesion, traumatic disc lesion, accidental injury of the spinal
cord, adhesive meningitis & retention of urine in patients with
senile prostate.
2. Cistemal Puncture :
Indications :
i. Failure of lumbar puncture or presence of local sepsis.
15
ii. Myelography.
Contraindications:
i. All contraindications for lumbar puncture.
ii. Suspected Amold-Chiari deformity.
3. Ventricular Puncture :
Indications :
i. Air ventriculography.
ii. Myodil ventriculography,
iii. To obtain CSP for analysis in iases of increased ICT.
iv. To reduce ICT temporarily in emergency e.g. impending tonsillar
heruiation.
The CSF Pressure:
* Normally 110—150
* Low pressure : below a complete spinal block
* High pressure in increased ICT & on abdominal compression.
The Queckenstedt Test:
* Pressing over the jugular veins on either side of the patient's neck, then on
both sides together-^increased CSF pressure. This is ineffective in patients
with complete spinal or foramen magnum block.
CSF Examination :
1. The Aspect :
- Normally clear & colourless.
- Cloudy C.S.F :
* Unclean apparatus
* High cell count 400/Cu mm.
* R.B. Cs → smoky C.S.F.
- Bloody C.S.F:
* in traumatic puncture.
* subarachnoid haemorrhage.
- Xanthochromic C.S.F. :
* in subarachnoid haemorrhage.
* high proteins e.g. below a complete spinal block, neurofibrom-atosis &
Guillain-Barre syndrome.
* Sometimes in subdural haematoma.
- Clotting C.S.F. :
16
* Traumatic puncture.
* Highly proteinous C.S.F. if contaminated by blood
* Cobweb clot in tuberculous meningitis.
2. The Cells:
i. Normally not more than 5 cells/cu mm.
ii. Polymorphonuclear Pleocytosis:
* Pyogenic bacterial meningitis → very high cells & low sugar.
* Leptospinal meningitis, acute haemorrhagic necrotising leucoencephalopathy & cerebral haemorrhage or infarction in contact with
the ventricles or brain surface → high cells & normal sugar.
iii. Both Polymorphonuclear & Mononuclear Pleocytosis : in tuberculous
meningitis, cerebral abscess, neurosyphilis & metastases.
iv. Mononuclear Pleocytosis :
Viral meningitis & encephalitis, tuberculous meningitis, very acute
demyelinative diseases, disseminated sclerosis, neurosyphilis & in
cerebral or spinal metastases.
3. The Protein Content - Normally 15 to 40 mg./lOO ml. It is lower in the cisterna magna & in the
ventricles.
- High protein content :
* below a complete spinal block.
* infective polyneuritis (L.G.B.S.)
* spinal neurofibromata.
* acoustic neuromata.
* cerebellar or spinal haemangioblastomata.
* sometimes in meningiomata, subdural haematomata & neurosyphilis.
- Excess of gamma globulins; in neurosyphilis, demyelinating diseases e.g.
D.S., Guillain-Barre syndrome & encephalitis.
4. The Sugar Content :
- Normally : 50—75 mg./l00ml.
- High sugar content in diabetes.
- Low sugar content in pyogenic & tuberculous meningitis
5. The Chloride Content :
- Normally : 700—750 mg./lOO ml.
- Low content in tuberculous meningitis & in low blood chloride.
6. The Colloidal Gold Curve (The Lange curve) :
- The addition of different dilutions of the CSF (10 dilutions) to colloidal
gold solution→ precipitation with colour changes (ranging in degree
from 5- to 0) due to the presence of abnormal globulins.
17
- Normally, no figure above 1 should appear.
- Three abnormalities are met with :
i. The first zone or paretic curve : The lowest dilutions the greatest
precipitation, as (5544321000)
Causes :
* G.P.I, & taboparesis (WR is+ve).
* D.S.
* Encephalitis & inclusion body encephalitis.
* Infective polyneuritis.
* Cysticercosis.
* Sometimes in spinal block.
ii. The midzone or luetic curve : as (0112332100).
Causes :
* Tabes dorsatis.
* Meningovascular syphilis.
iii. The end zone or the meningitic curve : e.g. in meningitis.
7. The Wassermann Reaction (W R) :
WR is+ve in G.P.I., glandular fever & me collagenoses.
8. Bacteriology & Virology : are important in pyogenic, tuberculous &
viral meningitis, encephalitis & meningo encephalitis.
N.B. : Bloody CSF after a traumatic L.P hite cell pleocytosis.
Air in CSF at e.g. air encephalography ononuclear pleocytosis.
II. NEURORADIOLOGY :
Localization of the lesion in the nervous system must be made accurately
on clinical grounds before x-rays are asked for.
A. PLAIN RADIOGRAPHY :
SKULL X-RAYING
Common views:
* Postero anterior with 20° tilt downwards.
* Lateral view.
* Towne's view.
* Basal view.
Indications :
* Head injury : fractures & displaced pineal body by I.C. haematoma.
* Suspected increased I.C.T. & other I.C. diseases.
* Systemic diseases with skull changes e.g. hypoparathyroidism.
* Congenital anomalies of the skull & hydrocephalus.
Abnormalities:
18
(1) The cranium :
* Diploic venous lakes in the parietal region are unimportant.
*Multiple rounded defects: in metastases, plyelomata & xanthomatosis.
* Single rounded defect: indiploic dermoid ; haemangioma of the skull,
burrhole & developmental detects.
* Beaten-silver appearance : in increased ICT.
* Pathological thickening: in Paget's disease, fibrous dysplasia of bone
& exostosing meningioma.
* Separated sutures: in increased ICT & head injury.
* Premature closure of the sutures : in craniostenosis.
(2) The Sohenoidal Ridges: (visualised through the orbit in P-A view).
* Unilateral thickening : in meningioma en plaque & fibrous dysplasia
of bone.
* Erosion of the superior orbital fissure : in meningiomata, optic nerve
gliomata, pituitary tumours & aneurysms.
(3) The Petrous Bones & Base of Skull : (in Towne's view & base).
* Erosion : in naso-pharyngeal tumours, glomus jugulare tumour,
acoustic neuromata, trigeminal neuromata & petrous epider-moids.
(4) The Basi-Occiput :
* Basilar invagination : developmental or 2ry to Paget's disease.
* Platybasia: flattened posterior fossa with increased basal angle.
(5) The Sella Turcica : (in lateral view)
* Erosion of the dorsum sellae : in increased ICT.
* Local destruction of dorsum sellae & clinoids : in supra sellar &
parasellar lesions e.g. cavernous aneurysms.
* Ballooning of the sella: in pituitary tumours, advanced increased ICT,
sphenoid carcinoma & chordoma.
(6) Jntracranial Calcification:
(a) Normal: e.g.
i. Calcified pineal body: (in Towne's view) in 55 % of normalpeople above 25 years of age.
ii. Displacement 21/2 mm. from the midline is pathological. It is
displaced to the side of the lesion in cerebral atrophy & to the
opposite side in space occupying lesions.
iii. Calcified choroid Plexuses: (in Towne^e view) two mottled
grape-like bodies.
19
iv. Calcified falx cerebri (A-P view): calcified linear or pear-shaped
shadow in the midline. It may be displaced in space-occupying
lesions.
(b) Pathological:
i. Calcified Neoplasms e.g. meningiomata, craniopharyngio-mata
& oligodendrogliomata.
ii. Calcified Angiomas → serpiginous speckled calcification.
iii. Calcified Aueurysms → A ring of calcification.
iv. Calcified Haematomata→ mottled calcification.
v. Calcified Tuberculomata → mottled calcification.
vi. Cerebral Atheroma e.g. acneroma of the carotid artery →linear
streaks.
vii. Calcified Basal ganglia & Dentate nuclei e.g. in toxoplasmosis.
SPINE X-RAYING :
Commoo views :
* lateral view.
* Antero-posterior view.
* Oblique view in the cervical spine.
Indications : in suspected spinal lesions e.g. spondylosis, paraplegia,
cauda equina lesion & brachial plexus lesion.
Abnormalities :
(a) Vertebral Bodies :
i. Collapsed vertebrae : in fractures, osteoporosis, metastases & Pott's
disease of the spine.
ii. Density is increased in Paget's disease, osteoblastic metastases,
sclerosing myeloma, reticulosis & Charcot's disease:
iii. Angioma → vertical trabeculation.
iv. Concavity of the posterior surfaces; e.g. in syringomyelia, intraspinal dermoids & cysts.
v. Hemi vertebrae: congenital & with lumbar disc lesion.
vi. Kypho scoliosis may cause paraparesis.
vii. Klippel Feil syndrome: Fusion of cervical vertebrae.
(b) The intervertebral disc spaces :
They are narrowed in degenerative disc lesion & in disc prolapse.
Usually associated with osteophytes.
(c) The pedicles : may be destroyed by metastases.
(d) The vertebral canal :
20
i. Narrowed : in spondylosis, achondroplasia, spinal deformity, basilar
impression & Paget's disease.
ii. Widened e.g. in dermoids & astrocytomata.
N.B.: The A-P diameter of mid cervical spinal canal is l7mm. If it
exceeds 22 mm this is diagnostic of syringomyelia.
(e) The intervertebral formina : (in. oblique views).
* The foramina are widened in multiple neurofibromatosis (von
Recklinghausen's disease).
* Osteophytes may encroach on the foramina in cervical spondylosis.
(f) Paravertebral shadows : are seen in T.B., reticulosis, myeloma,
haematoma & dumb-bell shape neurofibroma.
CHEST X-RAYING : (P-A view).
This is helpful to the neurologist in the following conditions:
i. Bronchogenic carcinoma →cerebral or spinal metastases.
ii. Pancoast tumour → brachial plexus compression.
iii. Bronchiectasis, lung abscess, empyema → brain abscess.
iv. Pulmonary T.B → T.B.meningitis, tuberculoma. Pott's disease.
v. Sarcoidosis → polyneuritis & polyneuritis cranialis.
vi. Mitralization → cerebral embolism.
vii. Emphysema may → papilloedema.
viii. Polyarteritis nodosa → focal neurological lesions.
ix. Thymoma in myasthenia gravis.
ABDOMEN X-RAYING:
i. Phaeochromocytoma.
ii. Hypemephroma.
iii. Cancer stomach & cancer colon (Barium meal & enema). These may
present with cerebral or spinal metastases.
PELVIS X-RAYING:
e.g. in Paget's disease & bone metastases.
JOINTS X-RAYING:
* Charcofs joints in the shoulder & knee e.g. in tabes dorsalis,
syringomyelia,... etc.
* Ankylosing spondylitis of the sacro-iliac joints may be associated with
sciatica or cauda equina lesion.
MUSCLES X-RAYING:
21
May show the calcification ofcysticercosis in patients with epilepsy.
NASO PHARYNX X-RA YING :
Encroachment of the air shadow may be seen in nasopharyngeal
carcinoma or lymphosarcoma which may present with cranial nerve
palsies.
B. CONTRAST RADIOGRAPHY :
CAROTID ANGIOGRAPHY :
Injection of a radio-opaque substance e.g. urographin into the common
carotid artery & taking serial films showing the arterial, capillary &
venous phases from one injection. Both the internal & external carotids
are visualized.
- Internal carotid may show : aneurysms, vascular anomalies & tumours.
- External carotid may show :
* Glomus jugulare tumours.
* Arteriovenous Fistulae.
* External carotid branches to a meningioma.
Main Views :
* Antero-posterior view.
* Lateral view.
* Oblique view : in suspected aneurysms.
* Each view needs a separate injection.
Indications :
i. Focal epilepsy at any age : to detect any tumour or vascular anomaly.
ii. Epilepsy starting above 30 years with focal onset, focal EEG changes
or focal paresis.
iii. Increased ICT : to detect supratentorial tumours.
iv. Head injuries : with hemiplegia, fits or impaired consciousness after a
lucid interval to detect subdural or extradural haematomata.
v. Spontaneous I.C. haemorrhage : to detect cerebral aneurysms or A-V
anomalies.
vi. Sudden hemiplegia in young adults with normal hearts to detect
angiomatous malformations.
vii. Recurrent hemiplegia : to detect stenosis or thrombosis of the internal
carotid.
viii. Chiasmal syndrome : to detect a pituitary adenoma i^id aneu-rysms.
ix. Sudden onset of unilateral 3rd or 5th nerve paLies, to detect vascular
anomalies.
Contraindications :
22
i. Old hypertensive & atherosclerotic patients unless it is life saving e.g. a
subdural haematoma or a meningioma.
ii. If no real indication e.g. in chronic headache.
VERTEBRAL ANGIOGRAPHY :
Injecting the radio-opaque material via a catheter passed through
femoral, brachial or axillary arteries to the vertebral artery under x-ray
screening control.
Indications :
i. To detect vascular anomalies in the domain of the vertebro-basilar
system.
ii. To investigate posterior fossa tumours.
iii. To differentiate between extrinsic meningiomata located anterior to
the brain stem & intrinsic brain stem gliomata. Both displace the
aqueduct & 4th ventricle back, but meningioma displaces the basilar
artery also backwards.
iv. To detect vertebral artery stenosis or occlusion.
Contraindications:
i. Old hypertensive & atherosclerotic patients.
ii. If not really & urgently indicated.
iii. History of-definite vertebro-basilar deficiency.
Abnormalities shown By Angiography :
1. Displacement of vessels : by tumours, cysts, baematomata, dilated
ventricles or brain oedema.
* Unilateral masses-^displacement of the pericallosal artery to the
opposite side.
* Unilateral atrophy-» displacement of vessels towards the same side.
2. Absence of vessels : may be due to spasm, thrombosis, embolism or
stenosis. Venous or sinus thrombosis-^non-filling of veins.
3. Arterial Stenosis : by atheromatous palques (e.g. carotid stenosis) or
constriction by a mass (e.g. sphenoidal ridge meningioma).
4. Angiomatous Malformations : e.g. Aneurysms & A—V fistulae may
cause I.C. haemorrhage, focal fits & cranial nerve palsies.
5. Tumours :
* Vascular Meningiomata -ablush. External carotid shares in its blood
supply. Drainage veins are dilated.
* Vascular Glioblastomata-^arterio-venous shunts & larger draining
veins.
* Vascular Metastases.
23
* Cystic Astrocytomata.
* Oligodendrogliomata, Brain abscess & I.C. haematoma-^Avascular
area displacing the normal vessels.
N.B.: Tiny tumours: infiltrafive tumours, thrombosis or haemorrhage
from minute vessels & some brain stem lesions may not be
shown by angiography.
AIR ENCEPHALOGRAPHY :
Introducing about 30 cc. of air through a lumbar puncture needle while
the patient is sitting & the head positioned to allow air to enter the
ventricles.
* The 4th ventricle & aqueduct are shown by films taken during the
filling stage.
* Then antero-posterior & lateral views (Brow up & Occiput-up
positions) are taken.
Indications :
i. Suspected cerebral atrophy.
ii. Suspected cerebral developmental anomaly.
iii. Low pressure hydrocephalus.
iv. Epilepsy starting after the age of 30
v.Sometimes in post-traumatic epilepsy.
vi. Tumours in the vicinity of the sella turcica not causing increased
ICT.
vii. Sometimes in acoustic neuroma without increased ICT.
viii. Suspicious angiography with no increased ICT.
Contraindications:
i. Signs of increased ICT for fear of conisation.
ii. Posterior fossa tumours or abscesses for fear of conisation.
iii. Stabilised congenital hydrocephalus.
VENTRICULOGRAPHY :
Introducing air or myodil into the ventricle through burrholes to visualize
the size, shape & position of the ventricles & the aqueduct. No fear of
conisation in patients with increased ICT.
Indications :
i. In posterior fossa & cerebello-pontine angle tumours.
ii. Failed angiography in patients with increased ICT.
iii. Suspected obstruction to 3rd or 4th ventricles or aqueduct.
Contra-LndicatioQs :
i. Local sepsis in the skull & scalp.
24
ii. Malignant hypertension with fear of cerebral haemorrhage.
iii. Cerebral abscess.
iv. In conditions that could be diagnosed by other safer techniques.
Abnormalities shown by Ventriculography :
1. Displacement & Deformity of the Ventricles e.g. by tumours, atrophy or
gliosis.
2. Filling Defects e.g. by intraventricular tumours.
3. Obstruction of the CSFflow e.g. obstruction at the foramen of Monro,
in the aqueduct, 4th ventricle or at the foramina of Luschka & Magendie.
4. Cerebral A trophy : →large pools of air over the cortex with or without
generalised ventricular dilatation.
5. Low pressure Hydrocephalus : → large ventricles, empty subarachnoid
space & no aqueductal obstruction.
6. Cerebral Developmental Anomalies : e.g. porencephaly, agenesis &
aqueductal atresia.
MYELOGRAPHY :
Oxygen under positive pressure, myodil or water soluble contrast media
may be used in myelography. Myodil myelography is the commonest of
all & is done by injecting myodil (6-9 cc.) through a lumbar or a cistemal
puncture into the subarachnoid space, tilting the patient under the screen
untill the site of the obstruction is visualised. Films are taken in the A-P &
lateral views.
Indications :
i. Focal cord lesions e.g. in paraplegia with a sensory level.
ii. Cervical spondylosis with cord compression.
iii. Kypho-scoliosis with pyramidal affection.
iv. Intramedullary lesions e.g. syringomyelia, astrocytoma, ependymoma
or angioma of the cord.
v. Suspected Amold-Chiari malformation (Here avoid cister-nal
puncture).
vi. Lumbar disc prolapse : for pro-operative accurate localization of the
lesion.
vii. Spinal block just before operative exploration.
Contra-indications:
i. Spinal block, unless operative exploration is sooner.
ii. Local sepsis.
iii. In patients exaggerating complaints, unless the indication is vital.
Abnormalities :
25
* Localization of the upper & lower limits of spinal block.
* Differentiation between extradural & intradural tumours.
* Diagnosis of intrinsic tumours of the cord.
* Angioma of the cord → serpiginous defects due to tortuous vessels.
* Disc prolapse → indentation of the myodil column.
* Arachnoiditis → irregular obstruction breaking up the myodil.
* Aroold-Chiari malformation → rounded defects below the foramen
magnum.
III. ELECTROENCEPHALOGRAPHY (EEG) :
Recording changes of electric potential in the brain by means of
electrodes applied to the scalp at different regions (frontal, parietal,
temporal & occipital).
Indications:
i.
Patients with attacks of impairment of consciousness e,g. epileptics.
ii. Sudden, unexpected episodes of behaviour changes e.g. in
psychomotor epilepsy.
iii. Children showing lapses of consciousness e.g. petitmal fits.
iv. Comatose patients.
v. Suspected intracranial infection e.g. cerebral abscess & encephalitis.
vi. Suspected hypoglycaemia.
vii. Unexplained intellectual deterioration e.g. in children with petit-mal
fits & subacute encephalitis.
viii. Follow up of patients recovering from a diffuse cerebral lesion e.g.
encephalitis.
ix. Diagnosis of brain death.
IV. BRAIN SCANNING (ISOTOPE ENCEPHALOGRAPHY) :
Certain radio-isotopes when administered orally or I. V. are picked up by
& concentrated in abnormal brain tissue but not in normal brain. These
isotopes emit gamma radiation which can be detected by a scanning
apparatus or by a gamma camera & recorded on a photographic film.
Technetium 99 m is the commonest isotope used & potassium
perchlorate is given orally prior to technetium to block uptake by the
choroid plexuses. The isotope is picked up by : meningiomas,
glioblastomas, metastases, haematomas, cerebral abscess & cerebral
infarction. Both False positive & false negative scans may occur.
V. ECHO-ENCEPHALOGRAPHY :
26
On direction of ultrasonic activity through the skull by an ultrasonic
probe, it will be reflected from the skull walls, ventricles & structures in
between them. The reflected echoes are recorded on an oscilloscope &
photographed. Displacement of midline structures to either side could be
detected. Both false positive & false negative echoencephalograms may
occur.
VI. ELECTROMYOGRAPHY (EMG) :
Recording the electrical activity of muscles, preferably by needle
electrodes introduced into the muscles & connected to a cathode ray
oscillograph & to a loudspeaker. A camera is included to photograph the
oscillographic patterns. EMG helps to differentiate between neurogenic &
myogenic lesions.
Indications of EMG :
i. Diagnosis & follow up of peripheral nerve lesions & facial palsy.
ii. Differentiation between neurogenic & myogenic lesions.
iii. Diagnosis of disorders of voluntary muscles & neuromuscular
junction e.g. myositis, myotonia, muscular dystrophies & myasthenia
gravis.
iv. Detection of carriers of muscle dystrophy e.g. in unaffected female
carriers of Duchenne type of muscular dystrophy (pseudohypertrophic type).
v. Localization of L.M.N.L.
N.B. : The Reaction of Degeneration (RD) :
Normally, faradic stimulation →sustained contraction, while
galvanic → single twitches on opening & closing the circuit.
Denervated muscle fails to respond to far adic stimulation & gives
an abnormally slow prolonged response to galvanic current,
VII. BIOPSY :
1. Brain biopsy : may be indicated in some disorders e.g. tumours,
dementia, encephalitis,... etc.
2. Peripheral nerve biopsy : to differentiate between leprosy,
hypertrophic polyneuropathy & amyloid infiltration.
3. Muscle biopsy : e.g. in collagenoses, muscular dystrophies & myasthenia gravis.
4. Liver, kidney, lung, skin, stomach, rectum, bone, or biopsy: may be
indicated to detect disorders that may be responsible for nervous
system
symptomatolgy
e.g.
liver
cirrhosis,
bronchogm€warcmnHia,...etc.
VIII. OTHER LABORATORY INVESTIGATIONS ;
27
1. Blood : RBCs, WBCs, Platelets, ESR, Blood urea. Blood sugar,......
etc.
2. Urine : Glycosuria, Bence-Jones Protein (in myelomatosis),
Porphyrins» Creatine & creatimne (in muscular dystrophy), Phytanic
add (in Refsum's disease), ......etc.
3. Faeces : for occult blood, parasitic ova, fats (steatorrheas),......etc.
IX. COMPUTERIZED AXIAL TOMOGRAPHY :
The introduction of CT scanning has revolutionized diagnostic practice of
intracranial lesions in the last few years.
Indications of CT scanning:
1. Long-standing epilepsy with only very slowly progressive signs:
* CT scanning can show in a non-invasive way the incidence & degree
of focal cortical atrophy & ventricular dilatation.
* It can exclude slow-growing cerebral tumours & hematomas.
* It can show about 70% of arteriovenous malformations (after i.v.
contrast enhancement).
2. Epilepsy of recent onset in an adult :
The possible causes include :
* Tumours e.g. meningiomas.
* Ischemia & infarcticm due to thrombo-embolic arterial disease or to
venous thrombosis.
* Arteriovenous malformations.
* Brain abscess & encephalitis.
* Subdural hematoma.
3. The recent onset of a condition in which clinical localization suggests
lesions disseminated in the CNS with or without evidence of raised
intracranial pressure:
The possible causes include:
* Multiple sclerosis.
* Acute encephalomyelitis following or accompanying systemic illness.
* Leukodystrophies.
* Multiple emboli whether presenting weith minor or major strokes or
with dementia.
* Multiple metastases.
4. Dementia either before or after the age of 65, with or without features
suggesting a vascular basis:
* CT can show in an atraumatic way the degree of cortical atrophy &
ventricular dilatation.
28
* It helps in the recognition of the cause of dementia e.g. low-pressure
hydrocephalus, multi-infarct dementia, frontal lobe tumours,....etc.
* It serves as an effective diagnostic screening of many patients for
whom pneumoencephalography was not justifiable.
5. Focal disturbances of sudden onset suggesting a vascular lesion with or
without impairment of consciousness:
* CT helps to separate hemorrhage from infarct & to distinguish infarct
from tumour.
* It is very helpful in differentiating subarachnoid hemorrhage from
primary intracerebral hemorrhage.
* Aneurysms & arteriovenous malformations can be shown by contrast
enhancement.
* Intracerebral or subdural hemorrhage, contusion & oedema may all be
shown in cases of a missed diagnosis of head injury.
6. Patients with clinical evidence suggesting intracerebral infection, with or
without well-defined focal signs, with a possible primary source:
* CT can exactly localize a brain abscess (acute or chronic) after
contrast enhancement.
* A tuberculoma is equally well shown by CT.
* Cerebritis without abscess formation & meningitis give few CT signs.
* Viral encephalitis gives uni- or bilateral cerebral oedema.
7. Patients with a progressive cerebral neurological disturbance with
clinical evidence suggesting raised intracranial pressure:
Possible causes include :
* Cerebral tumour e.g. gliomas & metastases.
* Extracerebral tumour e.g. meningiomas.
* Subdural hematoma.
* Chronic brain abscess.
8. Patients presenting with symptoms suggesting a lesion in the chiasmatic
cistern or the parasellar region such as a visual field defect or 3rd., 4th..
or 6th. nerve palsy:
* False negative scans are frequent.
* Clinical suspicion of a parasellar tumour with normal scan should lead
to an air-study & an angiogram.
9. Patients presenting with progressive cerebellar &/or brain-stem
dysfunction with evidence suggesting raised intracranial pressure:
The possible causes include:
* Intra- & extra-axial posterior fossa tumours.
29
* Developmental abnormalities e.g. basilar invagination & foramen
magnum stenosis.
* Infection e.g. cerebellar abscess.
Such patients usually need a combination ofCT scans & vertebral
angiograms for correct diagnosis.
Pneumoencephalography is indicated with any suspicion of
developmental abnormalities.
10. Patients presenting with progressive cranial nerve palsies with or
without brainstem or cerebellar signs:
* Cerebellopontine angle tumours include: acoustic neuroma &
meningiomas (enhance with contrast medium), metastases &
exophitic gliomas (need vertebral angiography), & epidermoids.
* Patients with lower cranial nerve palsies are difficult to diagnose by
CT & need other contrast studies.
30
NEUROLOGIC SYMPTOMATOLOGY
WEAKNESS
CAUSES OF WEAKNESS:
1. U.M.N.L. (Pyramidal Lesions) e.g. hemiplegia & paraplegia.
2. L.M.N.L. (Lower Motor Neurone Lesions) e.g. polyneuritis, Poliomyelitis & radiculitis.
3. Muscular Lesions e.g. :
* Muscular dystrophies.
* Myasthenia gravis.
* Polymyositis.
4. Extrapyramidal Lesions e.g. Parkinsonism → generalized weakness.
5. Hysterical weakness.
DIFFERENTIATION BETWEEN U.M.N. & L.M.N. LESIONS
Features
U.M.N.L.
L.M.N.L.
1. Paralysis or
Paresis :
2. Wasting :
­ On the contralateral side if the ­ Occurs in the muscles
lesion is above the pyramidal
supplied by the
decussation & on the
affected L.M.N.
homolateral side if the lesion
is below the pyramidal
decussation.
­ Absent (disuse atrophy
­ Present
sometimes occurs)
4. Reflexes :
­ Hypertonia (Spasticity).
­ Hypotonia (Flaccidity).
­ H/perreflexia(Exaggerated deep ­ Hyporeflexia or
5. Babinskis' sign :
­ Positive (Extensor plan tar
3. Muscle Tone :
tendon jerks)
areflexia
­ Negative.
response).
6. Fasciculation :
­ Absent.
7. Trophic changes : ­ Absent.
­ May be present.
­ Often present e.g. coldness, cyanosis, brittle
nails.
8. Reaction of
Degeneration
(R.D.) :
9. E.M.G.:
­ Absent.
­ Present.
­ No evidence of L.M.N.L
­ Evidence of L.M.N.L.
31
WASTING
CAUSES OF WASTING:
1. Muscle Diseases e.g. :
* Muscular dystrophies.
* Myotonia atrophica.
* Mysoitis.
2. Peripheral Nerve Lesions e.g. :
* Polyneuritis (infective, toxic, metabolic, ...etc.)
* Peroneal muscular atrophy.
* Ulnar, Median, Radial & External popliteal nerve injuries.
* Carpal tunnel syndrome.
3. Root Lesions e.g. :
* Spondylosis.
* Cervical rib.
* Trauma.
* Cauda equina lesion,
4. Anterior Horn Cell Lesions e.g. :
* Motor neurone disease.
* Poliomyelitis.
* Syringomyelia.
* Intramedullary tumours.
5. Parietal Lobe Lesions .
6. Disuse atrophy e.g. in the hand of old hemiplegics.
7. Rheumatoid arthritis.
8. Generalized wasting occurs in systemic diseases e.g. :
* Thyrotoxicosis.
* Malignancy.
HYPOTONIA
CAUSES OF HYPOTONIA:
1. Motor Lesions e.g. :
* Poliomyelitis.
* Polyneuritis.
2. Sensory Lesions e.g. :
* Tabes dorsalis.
* Herpes zoster.,
3. Combined Motor & Sensory Lesions e.g. :
* Syringomyelia.
32
* Cord or root compression.
4. Muscle Diseases e.g. :
* Muscular dystrophies.
* Myasthenia gravis.
* Benign infantile hypotonia.
5. Shock Stage e.g. :
* Acute hemiplegia,
* Acute paraplegia.
6. Cerebellar Lesions e.g.: Cerebellar ataxia.
7. Rheumatic Chorea
HYPERTONIA
CAUSES OF HYPERTONIA:
1. Pyramidal Lesions (ILM.N.L.): Spasticity of clasp-knife type.
2. Parkinsonism: Plastic or Lead-pipe Rigidity, or Cog-wheel Rigidity.
4. Reserpine or Chlorpromazine in high doses: Cog-wheel Rigidity.
5. Carbon monoxide poisoning: Cog-wheel Rigidity.
INVOLUNTARY MOVEMENTS
TYPES:
1. Convulsions :
* Grand mal epilepsy.
* Petit mal epilepsy.
* Myoclonic jerks.
* Jacksonian fits.
* Opisthotonos.
2. Extrapyramidal Lesions e.g.:
* Parkinsonism.
* Chorea.
* Athetosis.
* Dystonia.
* Hemiballismus.
* Spasmodic torticollis.
3. Cerebellar Lesions: intention tremors.
4. Lower Motor Neurone Lesions:
* Fasciculation (visible).
.* Fibrillation (invisible).
* Muscle spasm.
5. Psychomotor Disorders:
33
* Tics (Habit spasm) : in neurotics.
* Mannarism : in psychotics e.g. schizophrenics.
* Automatism : in epileptics.
PARKINSON'S DISEASE
Causes:
1. Arteriosclerotic Parkinsonism.
2. Post-encephalitic Parkinsonism.
3. Paralysis agitans.
4. Wilson's disease (Hepato-lenticular degeneration).
5. Post-traumatic Parkinsonism: rare.
6. M.S.
Criteria of Diagnosis:
1. Mask Expressionless fades with infrequent blinking.
2. Flexid Rigid Attitude of the limbs & trunk.
3. Akinesia or Bradykinesia with slowness of movements, slow, quite monotonous speech, diminution or loss of associated movements & facial immobility.
4. Muscular Rigidity affecting both progravity Sc antigravity muscles. Two
types arc known: plastic or lead-pipe type & cog-wheel rigidity.
5. Tremors: rhythmic, static, pill-rolling in U.Ls., flexion &. extension in
L.Ls.
6. Slow, shuffling, short-stepped gait ± propulsion, retropulsion & lateropulsion.
7. Sialorrhea, greasy face, oculo-gyric crisis & impotence may occur in the
postencephalitic type.
CHOREA
Causes:
1. Rheumatic chorea (Sydenham's chorea).
2. Huntington's chorea.
3. Hmiballismus.
4. Senile chorea.
5. Post-encephalitic chorea.
6. Hepatolenticular degeneration.
Clinical Varieties of Rheumatic Chorea:
1. Hemichorea.
2. Chorea gravidarum.
3. Chorea gravis
4. Maniacal chorea.
34
5. Paralytic chorea (Chorea mollis).
Criteria of Diagnosis:
1. Choreic Movements: involuntary, dysrhythmic, brief, nonstereotyped
movements in the face, mouth, tongue,U. & L.Ls. increased by excitement
& cease during sleep. ± Dysarthria.
2. Hypotonia with choreic posture of the hand.
3. Pendular knee-jerk, brisk superficial reflexes, & flexor plantar reflexes.
4. Intact sensations & sphincters.
5. ± Emotional instability.
6. ± Rheumatic heart disease.
ATHETOSIS
Causes:
1. Congenital bilateral athetosis (status marmoratus).
2. Congenital unilateral athetosis (usually with infantile hemiplegia).
3. post-encephalitic athetosis.
4. Arteriosclerotic athetosis.
5. Hepato-lenticular degeneration.
Criteria of Diagnosis:
1. Facial grimacing.
2. Athetotic movements: involuntary slow, dysrhythmic, snake-like movements affecting U.Ls. more than L.Ls., distal more than proximal.
3. Hypertonia during the movements & hypotonia during relaxation.
4. Dysarthria &. dySphagia.
5. Contractures, spastic diplegia, mental subnormality. & choreo-athetosis
may be present
DYSTONIA
Criteria of Diagnosis:
1. Dystonic Movements: involuntary very slow torsion movements of the
trunk & the proximal segments of the extrimities.
2. Hypertonia during the spasms & hypotonia between them.
3. Reflexes, sensations, mentality & speech are normal.
CEREBELLAR ATAXIA
Causes:
A. Hereditary Ataxias:
1. Friedreich's ataxia.
35
2. Mar'ie''s ataxia.
3. Sanger"Brown's ataxia.
4. The RouSsy'Levy Syndrome.
5. Progressive cerebeliar degeneration.
B. Symptomatic Ataxias:
1. Congenital e.g.:
* Cerebellar diplegia.
* Arnold-Chiary malformation.
* Basilar impression.
2. Traumatic.
3. Inflammatory e.g.:
* Encephalitis.
* Cercbellar abscess.
* Tuberculoma of the cerebellum.
4. Vascular e.g.:
* Cerebellar arteries occlusivc syndromes.
* Cerebellar haemorrhage.
5. Neoplastic e.g.:
* Vermal tumours: Medulloblastoma.
* Hemispherical tumours: Astrocytoma & Haemangioblastoma.
* Cerebello-pontine angle tumours: Acoustic neuroma.
6. Demyelinating e.g.:
* M.S.
* D.E.M.
7. Metabolic e.g.:
* Subacute cortical cerebellar degeneration of malignancy.
8. Toxic e.g.:
* Alcoholism.
* Chronic mercurial poisoning.
Manifestations of Neocerebellar Lesions in Man:
1. Nystagmus.
2. Slurred, staccato, scanning speech.
3. Hypotonia.
4. Pendular knee-jerk.
5. Ataxia:
i. Dysmetria with decomposition of movement.
ii. Intention tremors detected by the finger-to-nose & heel-to-knee tests.
iii. Adiadochokinesis.
36
iv. Rebound phenomenon.
v. Staggering, reeling, or drunken gait.
FRIEDREICH’S ATAXIA
Criteria of Diagnosis:
1. Cerebellar Manifestations: [Nystagmus + Slurred, explosive or scanning
speech + Intention tremors + Ataxic, broad base, reeling or staggering
gait].
2. Pyramidal Manifestations: (Weakness + Extensor plantar responses).
3. Posterior Column Affection: [Impaired sense of position, sense of
movement & vibration sense + Sensory ataxia (intension tremors &
unsteadiness of stance increase by eye closure)].
4. Peripheral Nerve Affection: [Peripheral muscular atrophy of the U. &
L.Ls. + hypotonia+Hyporeflexia orareflexia e.g. lost ankle jerks + Glove &
stocking hypoesthesia].
5. Skeletal Deformities & ECG Changes: [Pes cavus, kyphosis or scoliosis &
spina bifida].
MARIE'S ATAXIA
Criteria of Diagnosis:
1. Cerebellar Manifestations: [Intention tremors & ataxic gait],
2. Pyramidal Manifestations: [Weakness, spasticity, hyperreflexia & extensor
plantar responses],
3. ± Ophthalmoplegia & Optic atrophy.
4. No sensory changes or bony deformities.
TREMORS
Causes:
1. Nervousness & Anxiety states.
2. Benign essential familial tremors.
3. Thyrotoxic tremors.
4. Parkinsonian tremors (static tremors).
5. Cerebellar tremors (intention tremors).
6. Flapping tremors in pre-hepatic coma.
7. Hepato-lenticular degeneration (Wilson's disease).
8. Pseudo-athetosis or searching movements e.g. in:
* Tabes dorsalis.
* Cervical spondylosis.
37
* Carcinomatous sensory neuropathy.
9. Alcoholism.
10. Amphetamine Addiction.
11. Mercury Poisoning.
SENSORY IMPAIRMENT
Patterns:
1. Hemi-anaesthesia of the subcortical type e.g. in extensive lesions of the
thalannu. Usually associated with hemiplegia.
2. Hemianaesthesia of the cortical type e.g. in parietal lobe lesions or lesion
between the thalamus & the cortex as in cortical hemiplegia.
3. Hemihyperaesthesia or hyperalgesia e.g. in partial lesions of the thalamus.
4. Analgesia & thermo-anaesthesia of one side of the face & of opposite U.
&L. Ls. e. g. in lateral medullary syndrome as in vertebral artery
deficiency (Wallenberg syndrome).
5. Sensory level of hypoesthesia or anaesthesia e.g. in transverse lesion of
the spinal cord as in focal paraplegia.
6. Brown-Sequard Syndrome: e.g. in himisection of the cord → Ipsilateral
weakness (pyramidal lesion) & posterior column affection with
contralateral loss of pain & temperature sensation (spinothalamic lesion).
7. A Jacket of Dissociated Sensory loss: Loss of pain &. temperature
sensation over several segments with preservation of touch sensation e.g.
in intramedullary tumours & syringomyelia.
8. Loss of sensation of Saddle-shaped area ( S3,4,5 ) e.g. in :
* Cauda equina lesion: All sensory modalities are affected.
* Conus medullaris lesion: Touch is preserved.
9. Radicular Sensory Loss e.g. in :
* Posterior root lesions.
* Peripheral nerve lesions.
10. Glove & Stocking Anaesthesia e.g. in :
* Polyneuritis.
11. Loss of position & vibration sense (Posterior column lesions) e.g. :
* Tabes dorsalis.
* Friedreich's ataxia.
* Subacute combined degeneration of the cord (B^ deficiency).
* Carcinomatous neuropathy.
* Compression of the posterior aspect of the cord.
12. Patchy Sensory Loss e.g. in:
* Leprosy.
38
SENSORY ATAXIA
Causes :
* Polyneuropathy.
* Tabes dorsalis.
* M.S.
* Subacute Combined Degeneration, of the Cord.
* Friedreich's ataxia.
* Brown-Sequard syndrome.
* Benedict's syndrome.
* Thalamic infarction.
* Lesions of the Sensory Cortex.
Criteria of Diagnosis of Sensory Ataxia:
1. Impairment of deep pressure sensation, position & joint sense & Vibration
sense.
2. Hypotonia & Areflexia,
3. Positive Lhermitte's sign (Sudden flexion or extension of the neck → an
"electric shock" sensation which travels to the hands & feet): in lesions of
the posterior columns in the cervical region.
4. Positive Romberg's sign (Inability to maintain the upright position with the
eyes closed → swaying or falling).
5. Sensory ataxia with Stamping Gait.
6. Past-pointing is positive only enclosure of eyes.
GAIT DISORDERS
TYPES OF GAIT DISORDERS:
1. Circumduction Gait: in unilateral pyramidal lesion e.g. hemiplegia.
2. Scissors Gait: in bilateral pyramidal lesion e.g. Little's disease & Spastic
paraplegia.
3. Shuffling Gait (marche a petits pas) : in Parkinsonism.
4. Drunken Gait (Staggering Gait) : in Cerebellar ataxia.
5. Stamping Gait: in posterior column affection e.g. Tabes dorsalis.
6. High-steppage Gait: in foot-drop e.g. in polyneuritis.
7. Waddling Gait: in:
* Congenital dislocation of the hip joints.
* Pregnancy.
* Pelvic girdle muscle dystrophy.
* Vitamin D deficiency.
8. Limping: in painful conditions & bone diseases of L.Ls.
9. Hysterical Gaits: Bizarre, variable, not causing injury.
39
10. Involuntary Movements.
NEUROGENIC BLADDER
Innervation of the Urinary Bladder:
1. Sympathetic supply: (T11—L2)
* For bladder filling (motor to the internal sphincter & inhibitory to
detrusor muscle).
2. Parasympathetic supply: (S2—4.)
* For bladder evacuation (motor to detrusor muscle & inhibitory to. the
internal sphincter).
* Afferent fibres carry pain, touch, temperature & muscle stretch sensation.
3. Suprasegmental control :
* Bladder centre on the paracentral lobule on the medial surface of cerebral
hemisphere.
Its function is voluntary initiation or stopping of the act of micturition.
4. Pudendal nerve: Supplies the external sphincter.
Developmental Control of Micturition:
* In infancy: the bladder is evacuated reflexly (uninhibited bladder).
* At the age of one year: the bladder centre develops.
* Afferent impulses of bladder fullness ascend in the posterior columns.
* Efferent impulses for voluntary initiation of the act descend in the
pyramidal tracts. The act is completed reflexly.
NEUROGENIC BLADDER:
A. Lesion of sacral reflex arc:
1. Lesion of the afferent parasympathetic e.g. tabes dor salts → Sensory
atonic bladder (Retention, loss of sense of bladder fullness, loss of
voluntary control over micturition).
2. Lesion of the efferent (motor) e.g. Cauda equina lesion → Motor
atonic bladder, (Retention, preserved sense of bladder fullness, loss of
volitional control).
3. Lesion of both afferent & efferent e.g. Conus medullaris lesion →
Autonomous bladder (Incomplete, irregular, involuntary evacuation).
B. Lesions above the level of the Conus :
1. Pyramidal lesions → Precipitency.
2. Posterior column lesions → hesitency or sensory atonic bladder.
40
3. Complete transection of the cord → Automatic bladder (Regular,
complete evacuation).
C.Cerebral Lesions: e.g. Cerebral tumour or atheroma → Uninhibited
bladder.
RAISED INTRACRANIAL PRESSURE
CAUSES OF RAISED INTRACRANIAL PRESSURE:
1. Intracranial tumour or infection.
2. Obstructed CSF flow.
3. Hypertensive encephalopathy.
4. Hypcrcapnia (CO2 retention).
5. Pseudo-tumour cerebri:
* Thrombosis of intracranial venous sinuses.
* Oral contraceptives.
* Vitamin A poisoning.
THE FACIAL NERVE
Anatomy:
A. The Nuclear & Infranuclear Pathway (L.M.N.):
The motor nucleus of the facial nerve is located in the pons near the
midline → facial nerve fibres → a loop around the 6th nerve nucleus, then
emerge from the lateral surface of the pens → Internal acoustic meatus
(together with the nervus intermedius & the 8th. nerve) →facial canal (in
the petrous portion of the temporal bone) → geniculate ganglion (at the
angle of the facial canal) → emerge to the face through the stylomastoid
foramen → branches to the stylohyoid muscle, the posterior belly of
digastric & the occipital belly of occipitofrontalis. It then divides into 2
branches:
i. Temporo-facial → frontalis, orbicularis oculi & zygomatic.
ii. Cervico-facial → orbicularis oris, buccinator & platysma.
In the facial canal, the facial nerve gives the nerve to stapedius muscle
& chorda tympani.
B. The Sapraooclear Pathway (U.M.N.):
1. Corticospinal (Pyramidal) fibres ; for voluntary facial movement.
2. Mimic fibres (Extrapyramidal): for emotional & associative movements
of the face.
Functions of Facial Nerve:
41
1. Motor to: occipitofrontalis, orbicularis oculi, orbicularis oris, buccinator,
platysma, stapedius, stylohyoid & posterior belly of digastric muscle.
2. Secretory to submaxillary & sublingual salivary glands & to lacrimal
glands.
3. Sensations of external auditory meatus & preauricular area.
4. Proprioceptive sensations from the facial muscles.
5. Taste sensation from anterior 2/3 of tongue.
FACIAL PARALYSIS
CAUSES:
1. Supra-nuclear lesions e.g. cerebro-vascular accidents affecting the
pyramidal system (U.M.N.L.) &/or the extrapyramidal system (lesion of
mimic fibres).
2. Nuclear lesions e.g.:
* Pontine tumouis.
* Poliomyelitis.
3. Infra-nuclear lesions (L.M.N.L.)e.g.:

Cerebellopontine angle (CPA) space occupying lesions e.g. Acoustic
neuroma & meningioma.
 Bell's palsy.
 Parotid neoplasm.
 Trauma.
4. Disorders of facial muscles e.g. Facioscapulohumeral type of muscular
dystrophy & myotonia atrophica.
Causes of Bilateral Facial Palsy (Facial Diplegia) :
1. Congenital absence of facial nuclei.
2. Polyneuritis e.g. diabetic, diphtheritic, leprotic, ricoholic & acute infective.
3. Pontine lesions e.g. basilar artery thrombosis, brainstem encephalitis,
pontine tumours, .... etc.
4. Bilateral otitis media.
5. Syphilitic basal meningitis & carcinomatous meningitis.
6. Uveo-parotid syndrome of saicoidosis.
7. Double Bell's palsy.
Differential Diagnosis Between U.M.N. & L.M.N. Facial Palsy
ILM.N.L.
1. Paralysis :
L.M.N.L.
­ Muscles of lower 1/2 ­ Both upper & lower
of face
facial muscles.
42
2. Type of paralysis :
­ Voluntary movements ­ Both voluntary & emoare paralysed.
tional movements.
3. Hemiplegia :
­ Occur on the same
side if present.
­ Crossed hemiplegia i.e.
on the opposite side of
facial palsy.
4. Signs ofL.M.N.L.
­ Absent.
­ Present.
5. 5th,6th&ith. nerve
palsies :
­ Absent.
­ May be present
6. Taste loss over
­ Absent.
anterior 2/3 of tongue
7. Hyper acusis :
­ Absent.
­ May be present
­ May be present
BELL'S PALSY
A lower motor neurone paralysis of the facial nerve → Inability to corrugate
the forehead + inability to close the eye with widening of the palpebral
fissure + Deviation of the angle of the mouth to the opposite side on
attempting to show the teeth. + Inability to whistle. + Obliteration of the
naso-labial fold on the affected side. + Lost glabellar reflex ± Pain behind
the ear.
Aetiology: Any age, males > females, exposure to cold, virus of herpes
zoster, rheumatic interstitial neuritis, perineuritis orperiostitis, ischaemic
paralysis.
Clinical Picture:
* Unilateral or bilateral.
* Sudden onset with pain in the ear.
* Paralysis affects all facial muscles (emotional, associative & voluntary
movements are paralysed).
* Drooping of eyebrow.
* Loss of wrinkles in the forehead.
* Wide palpebral fissure.
* Inability to close the eye.
* Obliteration of the nasolabial fold on the paralysed side.
* Deviation of the angle of mouth to the sound side.
* Inability to retract the angle of mouth & to whistle.
* Accumulation of food between the teeth & cheek.
43
* Loss of taste over anterior 2/3 of tongue may occur.
* Hyperacusis may occur.
* Reaction of degeneration (R.D.) may occur.
Prognosis:
* Complete recovery in most cases.
* Contracture in paralysed muscles in some cases.
* Clonic facial spasm in some cases.
* Recurrent facial palsy is rare.
Treatment:
 Reassurance of the patient.
/ Hostacortin 5 mg Tab.
Or: Deltacortril 5 mg Tab.
(80 mg daily for 5 days, 60 mg on the 6th day, 40 mg on the 7th day, 30
mg on the 8th day, 20 mg on the 9th day, & 10 mg on the 10th day)
/ Acicone Susp.
Or: Epicogel Susp.
(1 tablespoonful before meals tid & 1 at bedtime)
± / Ranitidine 150 mg Tab.
Or: Zantac 150 mg Tab.
(2 tabs. at bedtime daily, if there is a history of peptic ulcer)
 Careful control of the blood sugar in diabetics.
/ Tears Naturale Oph. Solution Or: Visine Eye Drops
(Eye drops several times daily)
 An eye shield, or ointment to protect the cornea.
/ Terramycin Eye Oint.
Or: Miphenicol Eye Oint.
(Eye Oint at bedtime daily)
 Physiotherapy: Local heat massage, electrical stimulation. ± Surgery:
 Early Surgery: Surgical decompression was suggested, but medical
decompression by prednisone is preferable.
 Late Surgery: Plastic reconstructive surgery is indicated in persistent
severe cosmetic disability.
Other Medications:
/ Adenoplex Forte Amp.
Or: Adenophos Amp.
(1 amp. i.m. every other day)
/ Tri-B Double Amp.
Or: Trivarol Double Amp.
(1 double amp. i.m. every other day)
/ Mestinon 60 mg Tab.
Or: Mestinorm 60 mg Tab.
(1 tab. bid)
FACIAL PAIN
Causes:
1. Diseases of teeth, sinuses, ear, nose, or throat.
44
2. Trigeminal neuralgia.
3. Migrainous neuralgia (Cluster headache).
4. Post-herpetic neuralgia.
5. Atypical facial pain.
6. Temporo-mandibular arthritis.
7. Cervical spondylosis.
8. Acoustic neuroma.
9. Aneurysm of the posterior communicating artery.
10. Cranial arteritis e.g. temporal arteritis.
11. Myocardial ischaemia.
12. Superior orbital fissure syndrome.
PTOSIS
Causes:
1. Congenital ptosis.
2. Third nerve palsy
3. Homer's syndrome.
4. Myasthenia gravis.
5. Ocular myopathy.
6. Tabes dorsalis.
7. Hysterical ptosis.
NYSTAGMUS
Causes:
1. Physiological e.g. optokinetic nystagmus.
2. Errors of refraction & macular lesions.
3. Weakness of ocular muscles.
4. Cerebellar lesions.
5. Vestibular lesions.
6. Brain stem lesions.
7. High cervical cord lesions.
PSEUDOBULBAR PALSY
Definition:
Bilateral U.M.N.L. of the bulbar nuclei (9, 10, 11, 12).
Causes :
1. Vascular lesions e.g:
i. Double stroke (bilateral hemiplegia).
ii. Vertebro-basilar insufficiency.
45
iii. Cerebral arteriosclerosis.
iv. Syphilitic cerebro-vascular disease.
2. Motor neurone disease.
3. D.S. & D.E.M.
4. G.P.I.
5. Brain stem gliomas.
Clinical Picture:
1. Bulbar Manifestations :
* Dysarthria.
* Dysphagia.
* Hoarseness of voice.
* Nasal regurgitation of fluids.
2. Spastic Tongue.
3. Exaggerated jaw-jerk.
4. Emotional lability.
5. Quadriparesis or quadriplegia may be present.
PROGRESSIVE BULBAR PALSY
Definition:
Bilateral L.M.N.L. of the bulbar nuclei (9, 10, 11, 12)
Causes:
1. Motor neurone disease (Progressive bulbar palsy).
2. Poliomyelitis.
3. Syringobulbia.
4. Encephalitis.
5. Polyneuritis e.g. diphtheritic.
Clinical Picture:
1. Dysarthria.
2. Dysphagia.
3. Wasted fibrillating tongue.
4. Palatal paralysis.
HEMIPLEGIA
Causes:
I. Functional or Hysterical Hemiplegia.
II. Organic :
1. Congenital e.g.: Cerebral palsy.
46
2. Traumatic e.g.: Cerebral contusion, laceration, haemorrhage, subdural
haematoma, posttraumatic cerebral atrophy or cicatrization.
3. Inflammatory e.g.:
* Meningitis.
* Encephalitis.
* Neurosyphilis.
* Brain abscess.
4. Neoplastic e.g.:
* Brain tumour-Primary such as meningioma or glioma; or Metastatic.
5. Degenerative or Demyelinating e,g. :
* Multiple sclerosis (M.S.)
* Disseminated encephalomyelitis (D.E.M.).
6. Vascular Lesions: (The commonest cause of hemiplegia).
* Cerebral Thrombosis.
* Cerebral Embolism.
* Cerebral Haemorrhage.
* Hypertensive encephalopathy.
* Lateral sinus thrombosis secondary to otitis media.
Criteria of Diagnosis:
1. Paralysis or paresis of the upper & lower limbs on one side of the body.
2. Hypcrtonia (Spasticity of Clasp-knife type) after recovery from the shock
stage.
3. Hyperreflexia (Exaggerated deep reflexes) after recovery from the shock
stage.
4. Loss or diminution of superficial abdominal & cremasteric reflexes on the
hemiplegic side.
5. Extensor plantar response (Positive Babinski sign) on the hemiplegic side.
6. ± Hemianesthesia on the hemiplegic side.
7. ± U.M.N. 7th &1 or 12th nerve lesions on the hemiplegic side.
OR L.M.N.L. of 3rd, 6th, 7th, 9th, 10th, 11th, or 12th. cranial nerves on
the opposite side (Crossed hemiplegia).
8. ± Aphasia in left-sided lesions in right-handed people.
9. Circumduction Gait.
Localization of the Site of the Lesion in Hemiplegia:
I. Cortical Lesion: → Monoplegia &/or U.M.N. 7th ± Cortical sensory loss
± Jacksonian fits ± Aphasia 4: Coma ± Homonymous hemianopia.
II. Capsular Lesion: → Complete hemiplegia+ Subcortical hemianesthesia
± Homonymous hemianopia.
47
III.Midbrain Lesion: → Ipsilateral 3rd nerve paralysis+Contralateral hemiplegia.
IV. Pontine Lesion: →Ipsilateral 6th & 7th nerve palsies 4-Contralateral
hemi-plegia.
V. Medullary Lesion: → Ipsi lateral 9th, 10th, llth & 12th nerve palsies.
Homer's syndrome, Cerebellar ataxia & Trigeminal analgesia & thermoanesthesia + Contralateral Hemiplegia & Hemianesthesia.
VI. Spinal Cord Lesion (above C5): → Brown-Sequard syndrome
(Hemiplegia & Deep sensory loss on the side of the lesion 4- Loss of pain
& temperature sensations on the opposite side.
CAUSES OF TRANSIENT & RECURRENT HEMIPLEGIA:
1. Hypertensive encephalopathy.
2. Multiple sclerosis (M.S.).
3. Todd's paralysis (post-epileptic paralysis).
4. G.P.I. (General paralysis of the insane).
5. Hysterical hemiplegia.
6. Carotid hemiplegia.
7. Hemiplegic migraine.
PARAPLEGIA
Causes:
I. Focal Diseases → Paraplegia with a sensory level.
II. Systemic Diseases of the N.S. involving the pyramidal tracts.
III. Disseminated Diseases of the Nervous System.
I. FOCAL PARAPLEGIA:
A. Compression of the Spinal Cord e.g:
1. Diseases of vertebral column :
* Congenital : Kyphoscoliosis.
* Traumatic : Fracture & Fracture dislocation.
* Inflammatory :
 Spinal extradural or epidural abscess.
 Syphilitic spinal osteitis.
 Potfs disease (Tuberculous osteitis).
* Neoplastic :
 Secondaries in the spine.
 Primary vertebral tumours e.g. sarcoma, haem-angioma,
myeloma.
* Degenerative : Spondylosis with disc prolapse.
2. Extramedullary Tumours :
48
* Extradural e.g. :
 Angiomatous malformations.
 Malignant deposits.
* Dural e.g. :
 Meningiomas.
 Spinal pachymeningitis hypertrophica.
* Intradural e.g. :
 Neurofibromas.
 Arachnoid cysts.
3. Intramedullary Tumours :
* Ependymomas.
* Angiomas.
* Syringomyelia.
B. Inflammatory Causes :
1. Meningitis (Tuberculous, syphilitic, meningococcal).
2. Arachnoiditis.
3. Transverse myelitis.
C. Vascular e.g. : Anterior spinal artery occlusion.
II. SYSTEMIC DISORDERS OF N.S. CAUSING PARAPLEGIA :
A. Heredofamilial e.g. :
1. Hereditary spastic paraplegia.
2. Hereditary ataxias :
* Friedreich's ataxia.
* Marie's ataxia.
B. Cerebral Paraplegia :
1. Parasagittal meningioma.
2. Superior sagittal sinus thrombosis.
3. Thrombosis of an unpaired anterior cerebral artery.
4. Little's disease (Congenital cerebral diplegia).
C. Spinal Paraplegia due to :
1. Erb's syphilitic paraplegia.
2. Pellagral Lateral sclerosis.
3. Subacute combined degeneration of the cord (Bi2 deficiency).
D. Idiopathic or Degenerative e.g. ; Motor neurone disease.
III. DISSEMINATED DISEASES OF THE N.S. :
1. Multiple sclerosis (M.S.).
2. Disseminated encephalomyelitis (D.E.M.).
3. Disseminated syphilitic lesion.
4. Disseminated arteriosclerotic lesion.
49
Criteria of Diagnosis of Focal Paraplegia:
1. Paralysis or paresis of both L.Ls. of pyramidal distribution.
2. Spasticity of the Clasp-knife type in both L.Ls. after recovery from shock
stage.
3. Hyperreflexia (Exaggerated tendon jerks) in both L.Ls. after recovery
from the shock stage.
4. Loss or diminution of superficial reflexes below the level of the lesion
(abdominal & cremastcric reflexes).
5. Bilateral extensor plantar responses (Positive Babinski sign).
6. Sensory Level.
7. Precipitancy of micturition ± Constipation.
8. Spastic Gait.
9. ± Localized deformity, swelling or tenderness in the spine.
N.B.:
1. In Quadriplegia the same features are found, but affecting both U.& L.Ls.
2. In the Shock Stage paralysis is flaccid (Hypotonia) & all reflexes are lost
(Areflexia).
3. Paraplegia may pass through the following stages :
a) Shock stage: Common in acute lesions e.g. acute transverse myelitis &
anterior spinal artery occlusion.
b) Stage of Paraplegia in Extension: is due to gradual compression of the
pyramidal tracts.
c) Paraplegia in Extension passing into Flexion.
d) Stage of Paraplegia in Flexion: is due to interruption of the reticulospinal tracts as well as the pyramidal tracts.
CAUDA EQUINA LESION
Definition:
A lesion affecting the lumbosacral roots at any level from L1 to S 5.
Criteria of Diagnosis of Cauda Eqinua Lesion:
1. Atrophic paralysis (L.M.N.L.)of the muscles supplied by the affected roots,
usually in one L.L. or in both L.Ls. but asymmetrical. +Hypotonia &
hyporeflexia or areflexia e.g. Lost ankle-& or knee-jerks. + plantar reflexes
may be unelicitable.
2. Radicular sensory loss in the dermatomes supplied by the affected roots.
3. Retention of urine & faeces ± Impotence ifS3&4 roots are interrupted.
4. ± Positive Lasegue's & Kernig's signs.
50
CONUS MEDULLARIS LESION
Definition:
A lesion in the 3rd, 4th & 5th sacral segments of the spinal cord.
Criteria of Diagnosis:
1. Urinary incontinence.
2. Faecal incontinence.
3. Impotence.
4. Numbness & dissociated sensory loss in the saddle-shaped area.
5. No motor or sensory changes in the L.Ls.
EPICONUS LESION
Definition:
A lesion in the L 4,5 &. S1,2 Segments of the cord.
Criteria of Diagnosis:
1. Weakness in extension of hip, flexion of knee, & plantar-& dorsiflexion of
ankle.
2. Lost ankle-with preserved knee-jerks.
3. Sensory loss (may be dissociated) in the dermatomes supplied by L4.5 &
S1.2.
4. ± Precipitency of micturition.
5. ± Extensor plantar response.
N.B. Combination of Cauda, Conus & / or Epiconus Lesions may occur in
the same patient,
51
DISORDERS OF CONSCIOUSNESS & EQUILIBRIUM
COMA









Coma is a symptom of many life-threatening conditions that injure the
CNS. CNS dysfunction may be restricted to the cerebral hemispheres, the
brainstem, or both of them.
The cause of coma can be a toxic- metabolic process or a structural
lesion. The former usually produces coma without focal signs & the
latter produces coma with focal signs.
Toxic-metabolic processes & structural brain damage produce coma
through interrupting impulses from the ascending reticular formation (in
the brainstem) to the cerebral hemispheres. This makes arousal difficult
(in stupor) or impossible (in coma).
The comatose patient is unresponsive & unarousable.
The stuporose patient is only arousable by strong external stimuli.
Vegetative state refers to awake but unaware patients who sustained
severe brain damage & recover from the sleep-like state of coma, but
never interact meaningfully with their environment.,
Coma can be classified into two main types: Coma with focal signs. &
Coma without focal signs.
Coma With Focal Signs:
 Supratentorial: Cerebral hemorrhage & massive infarction.
subdural & extradural hematoma, cerebral tumor.
 Brain-stem: Pontine hemorrhage & infarction, cerebellar hemorrhage.
 Diffuse; Encephalitis, Head injury, Subarachnoid hemorrhage,
Electric shock, Hypertensive encephalopathy.
Coma Without Focal Signs:
 Diabetic ketoacidosis, Hypoglycemic coma
 Head injury e.g. concussion & brain edema
 Epilepsy.
 Drug overdose & intoxications.
 Hepatic failure.
 Renal failure.
 Hypothermia
 Myxedema
 Simmond's disease
 Adrenocortical failure
 Stokes-Adams attacks.
52
CAUSES OF COMA
Coma may be attributed to agranic causes or may be functional (hysterical) in
origin.
Organic
Causes:
I. Focal Cerebral Disorders.
II. General Systemic Disorders.
I. Focal Cerebral Disorders:
1. Traumatic e.g: head injury (Concussion, contusion, laceration,
haemorrhage, subdural haematoma)
2. Inflammatory: e.g.: Encephalitis, Meningitis, Cerebral abscess.
3. Vascular e.g. :
* Subarachnoid haemorrhage.
* Cerebral haemorrhage.
* Cerebral thrombosis.
* Cerebral embolism.
* Hypertensive encephalopathy.
4. Space-Occupying Lesions e.g.:
* Brain tumour.
* Brain abscess.
* Subdural haematoma.
5. Epilepsy (Post-epileptic coma).
II. General Systemic Disorders:
1. Toxic e.g.:
* CO poisoning.
* Acute alcoholism
* Barbiturates Poisoning.
* Salicylates poisoning.
* Opiates Poisoning.
* Other drugs: chlorpromazinc, chloral, Bromides, Datura & belladonna.
2. Metabolic e.g.:
* Uraemic Coma.
* Hepatic coma.
* Hyperglycemic coma.
* Hypoglycemic Coma.
* Porphyria.
* Hypothermia.
* Hyperpyrexia (Heat Stroke).
53
3. Endocrinal e.g.:
* Hypopituitary Coma.
* Hypocalcaemia.
* Hypercalcaemia.
* Myxoedema Coma.
* Addison's disease.
4. Fevers e.g.:
* Typhus.
* Typhoid.
* Malaria.
* Bacillary dysentery.
* Septicaemia.
* Cholera.
CAUSES OF COMA WITH FEVER
1. Pontine haemorrhage.
2. Cerebral malaria.
3. Encephalitis & meningitis.
4. Heat stroke.
5. Fevers.
6. Datura & atropine poisoning.
Diagnosis of coma depends on:

History: from the pt.'s friends, relatives & attendants.

Examination: for evidence of injury or intoxication & neurologic
deficits. Examination of the pupils, fundus, breath smell, respiration,
pulse, temperature, BP, neck rigidity, response to pin prick, tone, deep
reflexes & plantar responses.

Investigations: Urine (for sugar, acetone, protein & blood): Blood (Hb,
white blood count, urea, creatinine & glucose): CSF analysis of stomach
contents, skull x-raying, EEG, CT/MIR brain scanning, & X-ray chest.
Management of the Comatose Patient
I. Immediate Measures:
 Ensure an adequate airway, place the patient on his side with the foot of
the bed raised 15 to 45 cm. pull the tongue forward with fingers or
forceps, suction of secretions with a mechanical sucker, intubation &
artificial respiration.
 Stabilize the neck in case of trauma.
 Start resuscitative measures.
 Draw venous blood for stat determination of glucose, BUN, creatinine,
electrolytes. CBC, PT, PTT, LFTs, & osmolality.
54





Give an i.v. bolus of 50-100 cc dextrose 50% & maintain an i.v. line
Monitor respiration, blood pressure, pulse, rectal temperature & ECG
Draw arterial blood for stat ABGs.
Give naloxone (narcan) 0.4-0.8 mg i.v. & thiamine (vit. B1) 100 mg i.v.
Control seizures (or status epilepticus) with diazepam (valium) 5-10 mg
i.v. & phenytoin (epanutin) i.v. loading dose of 15-20 mg/kg (1000-1500
mg)
 Brief clinical assessment for signs of trauma, meningeal irritation, motor
& neuro-ophthalmologic examination.
 Treat any metabolic disturbance,
 Lumbar puncture, (LP.) in suspected cases of meningitis.
 If signs of herniation present: hyperventilate, give i.v. mannitol, obtain
CT brain scan and consult a neurosurgeon
II. Subsequent Measures:
 Insert nasogastric tube (NGT) & Foley catheter.
 Toxicologic screening of blood & urine.
 If seizures persist, give i.v. phenobarbital loading dose of 10-15 mg/kg
(750-1000 mg) or diazepam i.v. drip (100 mg diluted in 500 cc D5/W) at
a rate of 40 cc / hr.
 Radiography of chest, cervical spine, abdomen as indicated.
 CT scan of the brain to exclude space-occupying lesion (S.O.L) in
stabilized patients.
 If CT scan is negative, perform LP & CSF examination to exclude
subarachnoid hemorrhage (S.A.H.), herpes simplex encephalitis, hepatic
encephalopathy.
 EEG in suspected metabolic encephalopathy or seizures.
N.B.:
 Dextrose 50% reverses hypoglycemic coma.
 Flumazenil (Anexate) is a benzodiazepine antagonist.
 Naloxone (Narcan) reverses opiate-induced respiratory depression. &
CNS depression induced by barbiturates, benzodiazepines. & some
inhalation anesthetics.
 Thiamine (Vit. B1) reverses coma in Wernicke's encephalopathy.
 IV diazepam (Valium) is given at a rate not exceeding 2 mg/min until
seizures stop or to total of 20 mg.
 IV phenytoin (Epanutin) is given at a rate of 25-50 mg/min. to a total of
15-20 mg/kg. as a loading dose.
55

IV phenobarbital is given at a rate of 100 mg/min until seizures stop or
to a total 10-15 mg/kg.
 Diazepam (Valium) i.v. drip, 100 mg is diluted in 500 cc D5/W & given
at a rate of 40 cc / hr until seizures stop.
 IV Mannitol 20% is given rapidly over 15-20 min. in doses of 1-2 gm/kg
up to 500 ml, as a dehydration therapy.
Signs of herniation:
I. Central Downward Transtentorial Herniation:
A. Initial stage: (signs of failure of the hemispheres & subcortical
structures, usually reversible)
 Reduced consciousness.
 Small pupils, irreactive to light.
 Signs of bilateral pyramidal & extrapyramidal dysfunction
 Grasp reflexes
 Decorticate posturing.
 Periodic (Cheyne-Stokes) respiration.
B. Late stage: (marked by signs of midbrain failure, usually irreversible)
 Fixed, dilated or mid-position pupils.
 Decerebrate rigidity.
II. Uncal Herniation:
 Unilateral pupillary dilatation with a sluggish or absent reaction to
light.
 Deep coma from midbrain compression.
 Complete ipsilateral oculomotor nerve palsy.
 Contralateral decerebrate extensor posturing.
 NGT is inserted for gastric lavage to examine the stomach contents, &
for feeding. Comatose patients should be intubated prior to insertion of
NGT to avoid the risk of aspiration.
 Foley's Catheter is inserted to obtain a urine specimen for analysis &
toxicologic screening, & to collect urine for measuring its output & to
prevent bed wetting.
 Hyperventilation (by ambubag or mechanical respirator) helps reducing
ICP. PCO2 is reduced to 32 mm Hg or lower.
 Hypertension may point to increased ICP (Cushing reflex), brainstem
ischemia, or hypertensive encephalopathy.
 Hypotension may indicate Ml, hemorrhagic shock, sepsis, or sedativehypnotic drug overdose.
 Bradycardia with an elevated BP is a sign of brainstem compression
56

ECG may disclose acute MI, which may cause cerebral embolism or
anoxic ischemic coma due to cardiac arrest.
 Fever may indicate meningitis, S.A.H.
 Hypothermia may be attributed to overdose of sedatives, Wernicke's
disease, hypothyroidism & near-drowning.
 Depressed respiration may indicate overdose of opiates, sedative
hypnotics, or tranquilizers.
 Hypoventilation may follow overdose of tricyclic antidepressants,
anticonvulsants, & aspirin (after initial hyperventilation)
 Hyperventilation occurs in brain damage, SAH, & intake of acidotic
substances.
 Periodic (Cheyne-Stokes) respiration indicates bilateral hemispheric
dysfunction.
 Ataxic breathing indicates impending medullary failure & apnea.
 Obtain a CT scan before L.P., and avoid the latter if a cerebral mass
lesion is suspected or increased ICP is evident.
N.B.:
 Treatment of shock, Treating the underlying cause of coma, Care of the
patient's feeding, & Care of comatose patient should be ensured. An
adult should receive 2.5 litres of fluid (dextrose 5%) & 4.5 gm NaCl by
NGT or i.v. drip. Avoid CNS depressants in comatose patients as far as
possible.
 The depth of coma can be assessed according to Glasgow Coma Scale.
Comatose patient is given marks depending on his responsivity as
follows:
 Fully conscious individuals obtain 15 marks on "Glasgow Coma
Scale".
 Non-responsive patients obtain 3 marks.
 Other stages of coma lie inbetween
GLASGOW COMA SCALE
EYES
Open
BEST MOTOR
RESPONSE
No response
To verbal command
To painful stimulus
Spontaneously
To verbal command
To pain
Obeys
Localizes pain
Flexion-withdrawal
57
4
3
2
1
6
5
4
Flexion-abnormal (decorticate
rigidity)
Extension (decerebrate rigidity)
No response
Oriented and converses
Disoriented and converses
Inappropriate words
Incomprehensible sounds
No response
BEST VERBAL
RESPONSE
TOTAL
3
2
1
5
4
3
2
1
3-15
SYNCOPE
Classification of Syncope:
1. Vasodepressor syncope: Common fainting
2. Syncope associated with heart dysfunction
3. Reflex syncope: syncope associated with abnormalities of cardio-neural
reflexes
4. Syncope associated with metabolic abnormalities
5. Hypoxic syncope
6. Syncope associated with drug use
7. Syncope associated with specific neurologic disease
Cerebral Mechanisms in Syncope:
 Syncopal symptoms occur if oxygen, glucose, & cerebral blood flow are
diminished.
 20% of cardiac output goes to the brain which constitutes 2% of body
wt.
 Cerebral blood flow is approximately 750cc / min.
 The sole energy source of the brain is glucose
 The brain requires 3.3cc/100 gm of organ weight per minute of oxygen.
 Syncope is associated with a marked fall in mean arterial BP, reduction
in cerebral blood flow (CBF) & cerebral oxygen delivery.
Vasopressor Syncope (Common Fainting)
 Hypotension, sweating, nausea, sensation of coldness & warmth, &
generalized weakness ± Palpitations ± Giddiness + Black-outs + Loss of
consciousness.
 Convulsions ± Urinary incontinence may occur in some cases.
 Cardiac monitoring & echocardiography should be done in patients with
repeated faints.
58
 EEG may help differentiating fainting from seizure disorders.
 Patients should be advised to lie flat at the onset of symptoms.
Syncope Associated with Heart Dysfunction
 Hypertension, MI, CHF, valvular heart disease, & cardiac arrhythmias.
 Routine ECG & Cardiac monitoring are indicated
 Echocardiography discloses valvular disease, & cardiomyopathy.
 Cardiac catheterization may be required
Treatment is that of the underlying heart disease.
Syncope Associated with Abnormalities of Cardioneural Reflexes (Reflex
Syncope)
Examples:
 Hypersensitive carotid sinus (Carotid sinus syncope)
 Cough syncope
• Micturition syncope
Mechanism:
 Visceral distension or emptying, compression of baroreceptor areas on
blood vessels, or transient increases in venous pressure produce reflex
bradycardia &/or widespread  of peripheral vascular resistance.
Treatment:
 Micturition syncope: Patients are instructed to empty their bladder while
seated.
 Carotid-sinus syncope: Avoiding pressure upon carotid arteries.
 Cough syncope: Restraint of coughing
Syncope Associated with Metabolic Abnormalities
 Hypoglycemia, chronic hyponatremia, hypokalemia.
 Blood glucose & serum electrolytes should be measured in syncope.
 Correction of blood glucose level & electrolyte imbalance is required
Hypoxic Syncope
 It may occur in chronic lung disease producing hypoxemia.
Syncope Associated with Drug Use
 Tranquilizers, analgesics, hypnotics, & cardiologic agents may cause
syncope.
 History of drug intake & a drug - toxicologic screening of blood & urine
disclose the diagnosis.
 Postural hypotension is frequent among chronic drug users.
Syncope Associated with Specific Neurologic Disease
 Syncope may occur with migraine. Intracranial tumors, centrally
mediated postural hypotension, Parkinsonism, TIAs, posterior fossa
tumors, aqueduct obstruction.
59

EEG & CT brain scanning should be done.
VERTIGO

The vestibular apparatus is concerned with the sensation of linear
acceleration (related to the pull of gravity), & angular acceleration
 (related to rotations of the head) through two sensory organs, the otolith
organs, & the semicircular canals respectively.
 When the body's center of gravity is shifted, vestibulospinal reflexes
produce postural readjustments to prevent falls.
 An otolith disturbance produces a sensation of tilt ft a feeling that the
body is moving through the environment.
 A semicircular canal disturbance produces a sensation of rotation of the
body ft the patient may suddenly fall to the ground e.g. in Meniere's
syndrome.
 When the head rotates, vestibulo-ocular reflexes produce postural
readjustments of the eyes to keep gaze stationary & ensure best vision.
Abnormalities of vestibulo-ocular reflex cause nystagmus & oscillopsia
(illusory movement of the environment).
 Valsalva maneuvers (coughing, sneezing, straining) or exercise may
provoke vertigo caused by a cranio-cervical junction anomaly or
perilymph fistula.
 Loud noises, swallowing, air travel, or diving may provoke vertigo
caused by perilymph fistula.
 Hyperventilation, exercise, or hypothermia may provoke vertigo in
demyelinating diseases.
 The vegetative symptoms associated with vestibular diseases (e.g.
nausea, vomiting, prostration) are explained by two mechanisms:
1. An unusually prolonged vestibular stimulus.
2. The reception of conflicting information from the vestibular, visual,
& proprioceptive senses about the position &/or motion of the head
& body.
 Vegetative symptoms are usually less severe with central than with
peripheral vestibular lesions.
Treatment includes:
1. Symptomatic therapy of associated vegetative symptoms
2. Promoting rehabilitation of vestibulo-ocular & vestibulospinal function
to relieve vertigo, oscillopsia, & imbalance.
3. Treatment of the cause of vestibular disorder if amenable to therapy.
60
Acute Peripheral Vestibular Imbalance
1.
2.
Antiemetics to control vegetative symptoms
Encourage physical activity & start a series of vestibular training
exercises to promote rehabilitation & adaptation of vestibulo-ocular &
vestibulospinal reflexes.
/ Dramamine 50 mg Tab. (1 tab. every 4-6 hrs.)
Or: Phenergan 25 mg Tab. (1 tab. every 6 hrs. )
Or: Phenergan 50 mg Supp. (1 supp. bid)
/ Ephedrine HCl 30 mg Tab. (1 tab. every 6 hrs.)
If unresponsive, try:
/ Sinemet 275 mg Tab. (Half-1 tab. tid)
In vertigo associated with migraine, try:
/ Inderal 10 mg or 40 mg Tab. (10-80 mg tid)
In vertigo of obscure origin (? familial), try:
/ Diamox or Cidamex 250 mg Tab. (1 tab. every 6 hrs.)
61
Meniere's Syndrome
A. Medical Treatment:
/ Dramamine Tab.
Or: Phenergan Tab.
(1 tab every 6 hrs.)
/ Hygroton 50 mg Tab. Or: Hydrex 25 mg Tab.
(1 tab. daily)
+ Low-salt diet
B. Surgical Therapy: Endolymphatic drainage procedures.
 Labyrinthectomy when hearing is lost
 Vestibular nerve section when hearing is preserved
Benign Paroxysmal Positional Vertigo


It is thought to be caused by damage to the posterior semicircular canal.
Tilting the patient's head backwards elicits nystagmus (predominantly
vertical-torsional), vertigo, & nausea for a few seconds.
Treatment:
1. Physical therapy: 15-20 repetitions of a sequence of postural changes 34 times daily until improvement of vertigo.
2. A section of the ampullary nerve supplying the affected semicircular
canal is required in intractable cases.
NARCOLEPSY
A tetrad of symptoms, namely:
1. Excessive daytime sleepiness;
2. Cataplexy: a state of loss of muscle tone
Treated by:
/ Clomipramine (Anafranil) (25-100mg at night)
/ Flouxetine (Prozac) (20mg daily)
3. Sleep paralysis
4. Hallucinations in the hypnagogic (the state just before sleep) &
hypnopompic (the state just after sleep) states
 Onset: in the late teens
 A clear linkage to human lymphocyte antigen (HLA) type, HLA-DR2 &
DQR w1 on chromosome 6
 A pathognomonic feature of narcolepsy is the presence of 2 episodes of
daytime sleepiness with a REM onset (an episode of REM sleep within l0
min of falling asleep)
62
Diagnostic Criteria for Narcolepsy
 History of excessive daytime somnolence.
 Polysomnographic testing showing a mean sleep latency of less than 10
minutes on the Multiple Sleep Latency Test & the presence of at least
two REM onset sleep periods out of five possible situations (overnight
polysomnography plus the four nap periods of the test).
 History of at least one of the following accessory symptoms is suggestive
of the diagnosis:
1) Cataplexy,
2) Sleep paralysis, &
3) Hypnagogic/hypnopompic hallucinations
 If other disorders of sleep are present (i.e., sleep apnea or circadian
rhythm disturbance), they are treated before the diagnostic sleep studies.
 Psychotropic medications are discontinued at least 2 weeks before the
diagnostic sleep studies.
REM sleep deprivation, for example, because of discontinuation of the use of
drugs that suppress REM sleep, has been excluded.
Drug Treatment of Excessive Daytime Somnolence & Sleep Attacks in
Narcolepsy
Drug
Psychostimulants
Methylphenedate
Hydrochloride
(Ritalin)
Daily Dose
(mg)
Comments
50 – 60
Clinical effect lasts for 3 –6 h
Usually associated with fewer side
effects than dextroamphetamine.
Should be taken 30 – 45 min before
meals for proper absorption.
Methylphenedate
extended release
20 – 40
Clinical effect lasts for about 8h.
Dextroamphetamine
Sulfate (Dexedrine)
50 – 60
Clinical effect lasts for 3 – 6 h.
Dextroamphetamine
extended release
10 –40
Irritability & BP changes may limit
use.
Pemoline (Cylert)
37.5 – 112
63
Clinical effect lasts for 10 – 12 h
Anorexiant
Maxindol
Selegiline hydrochloride
(Jumex)
1–4
Structurally unrelated to
amphetamine, o.d. or bid
Liver function needs to be monitored.
10 - 30
Structurally unrelated to amphetamine
but with similar pharmacologic
effects.
Once or twice daily dosing with an
8 –15 h duration of action.
Should be taken 1 h before meals
N.B.:
(1) Drugs for Sleep Attacks include:
 Methylphenidate (Ritalin) 10 mg Tab.. 60 mg/day
 Methamphetamine (Methedrine) 2.5 & 5 mg Tab., 15-30 mg/day
 Dexamphetamine (Dexedrine) 5 mg Tab., 15-30 mg/day
 Pemoline (B-Alert) 20 mg Tab, 1-2 tabs. bid
 Ephedrine (Ephedrine HCl) 30 mg & 60 mg Tab.
 Caffeine (e.g. Coffee drinking)
 L-dopa + Carbidopa (Sinemet) 275 mg Tab.
 Methysergide (Deseril) 1 mg Tab., 2-4 mg/day
 Carbamazepine (Tegretol) 200 mg Tab.
 Propranolol (Inderal) 10, 40. 80 mg Tab 240 mg/day
 Codeine
 Phenmetrazine
(2) Drugs for Cataplexy:
 Tricyclic Antidepressants:
Imipramine (Tofranil) 10 & 25 mg Tab., 25-100 mg/day
Clomipramine (Anafranil) 10 & 25 mg Tab., 25-100 mg/day
Protriptyline (Concordin, Triptil) 5 mg & 10 mg Tab, 10-20 mg at
bed time ± 5 mg bid.
 MAO Inhibitors: should not be used with amphetamines or tricyclic
antidepressants. They are only indicated for patients unresponsive to
other drugs.
Phenelzine (Nardil) 15 mg Tab.
Tranylcypromine (Parnate) 10 mg Tab.
Nialamide (Niamid) 25 & 100 mg Tab.
64


Gamahydroxybutyrate (GHB), 1.5-2.5 gm at night + 1-1.5 gm with each
major re-awakening (intervals of at least 2 hrs between doses). It
promotes NREM & REM sleep.
Other Drugs:
Pemoline (B-Alert) 20 mg Tab.
Methysergide (Deseril) 1 mg Tab.
Clonidine (Catapres) 0.15 mg Tab.
AUTONOMIC DYSFUNCTION
Autonomic Dysfunction
Clinical Symptoms
(1) Abnormal BP control Weakness, fatigue.
• Orthostatic hypotension dimming / loss of vision,
• Supine hypertension
dizziness, syncope
Management
Practical suggestions
Physiological maneuvers
Pharmacological agents
(2) Neurogenic bladder
Frequency, dysuria,
infection
Urinary acidification ±
Antibiotics
(3) Decreased G.I.
motility
Constipation, impaction,
Bowel distension,
septicemia
Bowel training program:
dietary fiber bulk agent
suppositories
(4) Anhidrosis
Defective temperature
regulation
Avoid extremes of ambient
temp, use light dress
(5) Decreased lacrimation Conjunctivitis
Artificial tears
(6) Decreased salivation
Choking, cough syncope
Increase fluid intake
(7) Abnormal pupillary
responses
Blurred vision, diplopia
Special glasses, eye patch
Management of Orthostatic Hypotension
A. Practical Suggestions
 Get up slowly
 Avoid prolonged standing
 Avoid alcohol
 Remain in moderate temperature & avoid heavy dress
 Eat small meals
 Avoid excessive straining (e.g. at defecation & urination)
 Nonprescription drugs should be avoided
65
B. Physiological Maneuvers
 Increase salt & fluid intake
 Elevating the head of the bed at night reduce supine hypertension &
nocturnal diuresis
 Change posture frequently
 Compressive garments may help
 Pacemaker & sympathetic neural prosthesis are rarely used
C. Pharmacological Agents
 Volume expansion (Fludrocortisone)
/ Astonin H 0.1 mg Tab., (0.1 mg daily-up to 0.4 mg/day)
/ Slow-K 600 mg Tab.
Or: Addi-K 750 mg Tab
Or: Potassium Syrup, 750 mg / 5 ml
(1 tab, or 5 ml tid)
If unresponsive, discontinue gradually & try:
 Prostaglandin inhibition (Indomethacin, or Ibuprofen):
/ Indocid 25 mg Cap., (25-50 mg tid, 30 min. before meals)
Or: Brufen (200 mg or 400 mg Tab., or 600 mg tid)
If unresponsive, add:
/ Astonin H Tab. (1 tab daily, increase up to qid.)
 Beta-blockade (Propranolol):
/ Inderal (10 mg or 40 mg Tab, (10-40 mg qid)
 In acute hypotensive situations, use a Sympathomimetic e.g:
/ Ephedrine HCI 30 mg or 60 mg Tab. P.R.N.
Or: Vasopressin e.g.:
/ Minirin (DDAVP) Intranasal solution.
(Nasal spray P.R.N)
 In cerebellar dysfunction, try trihexylphenidyl ± amantadine:
/ Artane 2 mg Tab, (1 mg tid)
/ Adamine 100 mg Cap. (1 cap. tid.)
 Other Drugs:
 Presynaptic receptor inhibitor (Yohimbine)
 Post-synaptic receptor agonist (Clonidine: Catapres)
 Decrease norepinephrine metabolism:
¤ MAO inhibitor (Tranylcypromine: Parnate)
¤ Block neural uptake (Desipramine: Pertofran)
 Ganglionic stimulant (Caffeine)
 CNS stimulant (Methylphenidate: Ritalin)
 Vasoconstriction (Dihydroergotamine: Dihydergot)
66
PAINFUL SYNDROMES
MIGRAINE & CLUSTER HEADACHE
Migraine: is a paroxysmal headache, usually unilateral, throbbing in
character, accompanied by gastrointestinal disturbance (e.g. nausea &
vomiting) & hyperacuity of special senses ± preceded for 24 hrs by
premonitory changes in mood or appetite.
Varieties of migraine include:
(1) Classical migraine (Migraine with aura of visual hallucinations)
(2) Common migraine (Migraine without aura)
(3) Complicated migraine (Hemiplegic, Ophthalmoplegic, Basilar artery
migraine, Acute confusional state)
(4) Migraine variants (e.g. Cyclic vomiting).
EEG changes are observed in some migrainous patients.
CT Scan of the brain is indicated in complicated migraine.
Cluster Headache:
Three forms are identifiable:
(1) Episodic cluster headache (recurs in bouts)
(2) Chronic cluster headache or chronic migrainous neuralgia (regular
attacks without remission)
(3) Chronic paroxysmal hemicrania (brief attacks recur < 6 times daily)
Migraine Abortive Therapy
Drug
Paracetamol
(Panadol)
Dosage
1–4g
Acetylsalicylic
acid (Aspirin)
Diclofenac
(Cataflam)
1–3g
Ibuprofen (Brufen)
Ergotamine
(Migril,
Migrainil,
Cafergot)
Dihydroergotamine (Dihydergot)
600-1200 mg
Oral: 1-2mg
Rectal: 0.51.5mg
50 –150 mg
Adverse effects
Allergy, anemia,
renal & hepatic
toxicity
Allergy, asthma,
GI bleeding
Vomiting, GI
bleeding,
hepatotoxicity
Contraindications
Hepatorenal
insufficiency,
alcoholism, allergy
GI ulcers, allergy,
asthma
GI ulcers, allergy,
pregnancy
Nausea, vomiting,
cephalalgias,
ergotism
Coronary pathology,
Raynaud's; disease,
pregnancy;
association with
sumatriptan, methysergide, beta-blockers
or erythromycin
SC: 1-3 mg
Nasal: 1-4 mg
67
Sumatriptan
(Imigran)
SC: 6-12 mg
Oral: 50-300 mg
Zolmitriptan,
(Zomig)
Naratriptan,
(Naramig)
Rizatriptan,
(Maxalt)
Nasal: 20-40 mg
2.5 mg tab.:
2.5-5 mg
2.5 mg tab.:
2.5-5 mg
5 mg & 10 mg
tab.: 5-10 mg
Local pain,
paresthesia,
vertigo, flushing,
chest oppression
Nausea,vomiting,
dysgeusia, thoracic
oppression
Dysgeusia, nausea
Coronary pathology,
non-treated TIAs;
association with other
vasoconstrictors,
MAOIs, Lithium
Migraine Prophylactic Therapy
Drug
ß – Blockers
Propranolol
Inderal
Inderal LA
Timolol maleate (Blocadren)
Nadolol (Corgard)
Atenolol (Tenormin)
Metoprolol (Lopressor)
Enalapril (Renitec)
Calcium Channel Blockers
Verapamil (Isoptin)
Nimodipine (Nimotop)
Flunarizine (Sibelium)
Alpha Agonist
Clonidine (Catapres-TTS)
Antidepressent
Amitriptyline (Tryptizol)
Imipramine (Tofranil)
Sertraline (Lustral)
Fluoxetine (Prozac)
Phenelzine (Nardil)
Isocarboxazid (Marplan)
Dosage
80-240 mg daily in
divided doses
60-160 mg o.d.
5-30 mg daily
40-80 mg daily
50-100 mg bid
50-100 mg bid
10-40 mg daily
30-60 mg daily
30-60 mg daily
5-10 mg daily
0.1 mg tid
50-100 mg every
night
10-150 mg
50-200 mg
20-60 mg
15 mg tid
10 mg qid
68
Adverse Effects
Fatigue, lassitude,
depression, insomnia,
nausea, vomiting,
constipation.
Headache, hypotension,
flushing, edema, constipation.
May aggravate atrioventricular
nodal heart block & CHF.
Dry mouth, drowsiness,
sedation, headache,
constipation.
Sedation, dry mouth,
constipation, wt gain, blurred
vision, edema, hypotension,
urinary retention.
Anxiety, insomnia, sweating,
tremor, GIT disturbances.
Platelet Antagonists
Aspirin (Aspirin)
Sulfinpyrazone (Anturan)
Dipyridamole (Persantin)
NSAIDs
Naproxen (Naprosyn)
Ketoprofen (Orudis)
Fenoprofen (Nalfon)
Tolmetin sodium (Tolectin)
Miscellaneous
Cyproheptadine (Periactin)
Ergotamine tartrate with
phenobarbital & Bellafoline
(Bellergal-S)
Methysergide maleate
(Deseril)
325-650 mg per day
200 mg bid
50 mg qid
250 mg tid
75 mg tid
300mg qid or
600mg/d
200 mg tid
4-16 mg daily
One tablet bid
2 mg tid
Dyspepsia, gastrointestinal
bleeding.
GIT symptoms
Skin rash
Hypersensitivity reactions
Sedation, dry mouth,
epigastric discomfort,
gastrointestinal disturbances.
Nausea, vomiting, abdominal
pain, diarrhea.
Nausea, vomiting, diarrhea,
abdominal pain, cramps,
weight gain, insomnia,
edema, peripheral
vasoconstriction.
Retroperitoneal &
pleuropulmonary fibrosis &
fibrous thickening of cardiac
valves may occur.
Hepatic impairment
GIT symptoms
Skin rash
Hypersensitivity reactions
Valproic acid (Depakine)
500-1000 mg daily
Menstrual Migraine
600 mg tid*
Therapy
Fenoprofen calcium (Nalfon)
Naproxen sodium
250 mg tid*
(Naprosyn)
Mefenamic acid (Ponstan)
250 mg bid or tid*
*These drugs should be started 2 days prior to menses & continued through menses.
Therapy of Cluster Headaches
Prophylactic Treatment
Drug
Methysergide maleate (Deseril)
Prednisone (Hostacortin)
Methylprednisolone
Dosage
2 mg tid p.o.
40 mg per day in divided doses (to be tapered
slowly) p.o.
16 mg every other day p.o.
69
(Medrol, Urbason Retard)
Ergotamine tartrate with
phenobarbital & Bellafoline
(Bellergal-S)
Lithium carbonate (Priadel)
Verapamil (Isoptin)
Abortive Treatment
Oxygen 100%
Dihydroergotamine (D.H.E)
Lidocaine 4% (Xylocaine)
Cocaine HCl 10%
Ergotamine tartrate with caffeine
(Migrainil, Cafergot)
1 tablet bid p.o.
300 mg tid (serum levels should be monitored
to prevent toxicity) p.o.
240 mg per day p.o.
Inhalation by face mask, at 7 L/min for 15
minutes PRN
Intranasal solution 1 ml at hourly intervals up
to 3 ml per day
Intranasal solution 1ml (15-16 drops) to affected
nostril, repeat in 5 mins PRN, max. qid.; use
nasal drops in case of nasal ongestion
2 drops into affected nostril or both nostrils, 4
times daily PRN
2 tablets immediately at onset, repeat every ½
hour up to 6/ day
Insert 1 supp. at onset, repeat in 1 hour if
necessary up to 2supp./d
TENSION – TYPE HEADACHE
Criteria of Diagnosis of Tension-Type Headache
A. Episodic Tension – Type Headache
 Recurrent episodes of headache lasting minutes to days
 The pain is typically pressing/tightening in quality, of mild or
moderate intensity, bilateral in location & does not worsen with
routine physical activity
 Nausea is absent, but phonophobia may be present
B. Chronic Tension – Type Headache
 Headache present for at least 15 days a month during at
least 6 months
 The headache is usually pressing/tightening in quality, mild or
moderate in severity, bilateral & does not worsen with routine
physical activity
 Nausea, photophobia or phonophobia may occur
C. Headache of the Tension – Type not fulfilling the above Criteria
D.D. of Tension – Type Headache
1. Migraine
70
2.
3.
Cluster headache
Headaches secondary to other causes e.g. subarachnoid hemorrhage,
meningitis, temporal arthritis, increased ICP, hypertension, …etc.
Treatment:
 Non-narcotic analgesics & muscle relaxants for 2 weeks
/ Ponstan forte Tab.
Or: Panadol Extra Tab.
Or: Ketofan 50 mg Tab.
Or: Brufen 600 mg Tab.
(1 tab prn)
/ Norgesic Tab.
Or: Relax Cap.
(1 tab. or 1 cap tid)
 Treatment of the underlying cause
 Relaxation exercises, neck or back massage & biofeedback
 Antidepressants for depressed patients
/ Tryptizol 25 mg Tab.
Or: Tofranil 25 mg Tab.
(1-2 tabs. at bedtime daily)
 Psychiatric consultation
HEADACHE PRESENTING IN EMERGENCY DEPARTMENT
SAH
Meningitis
Temporal
Arteritis
Acute or
Chronic
Acute or
Chronic
Acute
Acute
HTN
Migraine
Cluster
Tension
type
Chronic
Onset
Acute
Acute or
Chronic
Location
Global
Global
Localized
Occipital
Frontal
Unilateral
Unilateral
Global
Unilateral
Associated
Symptoms
Nausea,
vomiting
LOC,
Meningismus,
focal
neurological
symptoms
Nausea,
vomiting,
fever,
photophobia,
meningismus,
focal
symptoms
seizures
Weight
loss,
fever,
Decreased
vision,
jaw
claudication
Nausea,
vomiting
focal
neurological
symptoms
Nausea,
vomiting
Photophobia,
Phonophobia,
Rhinorrhea,
Lacrimation
of
ipsilateral
side
Multisomatic
complaints
Characteristic
pain
Worst
ever
Severe
throbbing
over area
affected
Severe
throbbing
Throbbing
Throbbing
Sharp,
stabbing
Ache
71
Duration
Prior
History
Diagnostic
Tests
Brief
Brief
-
-
CT 8090%
LP(+),
CBC
Prolonged
-
Brief
+
Prolonged
+
30
minutes to
2 hrs
+
Daily
+
ESR (+)
CT scan
to rule
out
bleeding
Physi+ Focal
MeninTender
PapilleNausea,
Unical
signs,
gismus
temporal
dema
vomiting
lateral
Exami+
decreased
arteries,
decreasPhotorhinonation
decreased
LOC,
myalgias
es;
phobia,
rrhea
LOC,+
irritability
fever
venous
Phonolacrimenin& rash
pulsation
phobia
mation
gismus
decreaspartial
ed; LOC,
CV
changes
LOC = Loss of consciousness; LP = Lumbar puncture; CBC = Complete blood count;
CV = Cardiovascular; ESR = Erythrocyte sedimentation rate
Treatment of Headache Presenting in the Emergency Department
Drug
Dosage
1 mg IM
Dihyroergotamine mesylate
(D.H.E. 45)
1 mg slow
IV
Comment
Up to 3mg/day, 6mg/wk ± with
antinauseant, such as
metoclopramide (Primperan) 10
mg IM
Up to 2 mg/day, 6 mg/week
Sumatriptan (Imigran)
6 mg, SC
6 mg may be repeated after one hr.
Maximum 12 mg in 24 hours
Ketorolac (Toradol)
6 mg, SC
6 mg may be repeated after one hr.
Maximum 12 mg in 24 hours
Ketorolac (Toradol)
60 mg IM
Up to 120 mg per day; no more
than 3 injections per week
Chlorpromazine
(Largactil, Neurazine)
1 mg/kg IM
50-100 mg IM every 4 hours, no
more than 2 injections in 24 hrs
72
Naproxen sodium (Naprosyn)
500-750 mg
PO
25 mg / 4 hrs up to 1250 mg/day
Ketoprofen (Orudis)
100 mg PO
50 mg / 4 hrs up to 200 mg/day
Ibuprofen (Brufen)
1600 mg PO
800 mg / 4 hrs up to 3200 mg/day
Chlorpromazine
(Largactil, Neurazine)
100 mg
rectal
Every 6 hours, PRN
Status Migrainosus
Dihyroergotamine mesylate
(D.H.E. 45)
1mg slow IV
Chlorpromazine
(Largactil, Neurazine)
1 mg/kg slow
IV
Dexamethasone acetate
(Decadron LA)
16 mg IM
Dexamethasone (Decadron )
1.5 mg PO
Every 8 hours up to 2 mg/day,
6 mg/week. Many precede each
dose by an antinauseant, such as
metoclopramide (Primperan) 10
mg IM
Every 12 hours
Not to be repeated in < 3 weeks
BID for 48 hours, not to be
repeated in < 3 weeks
TRIGEMINAL NEURALGIA
Trigeminal Neuralgia (Tic Douloureux): momentary, electric-shock-like,
lancinating pains, with abrupt onset & sudden termination, along the
distribution of one or more of the divisions of the trigeminal nerve, usually
the 2nd, & 3rd divisions, commonly triggered by non-noxious stimulation
(e.g. touching the trigger area, chill, mastication, swallowing,... etc.) ±
hyperesthesia in the painful area, otherwise no neurological abnormalities. It
is most common in the elderly, & more common in women than men.
Glossopharyngeal Neuralgia: neuralgic pain in the distribution of the
glossopharyngeal nerve.
Criteria of Diagnosis of Trigeminal Neuralgia
 Trigeminal neuralgia is a painful unilateral affection of the face,
characterized by brief electric shock-like (lancinating) pain limited to
the distribution of one or more divisions the trigeminal nerve.
 Pain is commonly evoked by trivial stimuli including washing, shaving,
smoking, talking & brushing the teeth, but may also occur
spontaneously.
73

The pain is abrupt in onset & termination & may remit for varying
periods.
Treatment:
A. Drug Therapy:
Drug
Dose
Carbamazepine (Tegretol)
200 – 600 mg
Phenytoin (Epanutin)
200 – 400 mg
Gabapentin (Neurontin)
400 mg t.i.d
Amitriptyline (Tryptizol)
25 - 75mg / day
Baclofen (Lioresal)
30 – 80 mg
Adverse Effects
Blood disorders
CNS, hemopoietic, gum
hyperplasia
Somnolence, fatigue,
dizziness & wt gain
Sedation, atropine-like
effects
Drowsiness, weakness,
nausea, vomiting
N.B.:
 Morphine 10 mg i.m. may be used if necessary to ameliorate pain
 Blocking the trigeminal or glossopharyngeal nerve in the trigger zone or
the painful area can provide short-term relief. Local infiltration
anesthetics are used for this purpose.
B. Surgical Therapy:
Procedure
Glycerol injection
Benefits
85% effective
No craniotomy
Minor procedure
Radiofrequency rhizotomy
90% effective
Minor procedure
Brief hospitalization
Microvascular
decompression of the
trigeminal root
90% effective
No sensory loss
Complications
Masseter weakness
Facial sensory loss
Facial sensory loss
Facial weakness
Corneal hypoesthesia
(10-15%)
Major craniotomy
± 4% critical
postoperative
complications
1% mortality
Prolonged hospitalization
NB:
 Trigeminal neuralgia secondary to a vascular anomaly or a tumour in the
posterior fossa needs surgical treatment. Multiple sclerosis (M.S.) may
74
present with trigeminal neuralgia & should be properly managed to
alleviate pain.
LOW BACK PAIN & SCIATICA
Low back pain, increased by movement, relieved by rest, associated with
lumbar muscle spasm, tenderness, & straightening of the normal lumbar
lordosis. Straight leg raising test is positive (produces sciatic pain) if the
nerve roots are compressed e.g. in disc herniation. Sciatica is pain radiating
down the back of the leg along the distribution of the sciatic nerve, intensified
by coughing, sneezing, & straining. Root compression produces motor,
sensory, & reflex changes in the leg & foot. CSF proteins are raised. Plain xrays, CT scan-myelogram, Magnetic Resonance Imaging (MRI) of L-S spine,
Pantopaque myelography. & Electromyo-graphy (EMG) confirms the
diagnosis.
Treatment:
A. Conservative Management:
1. Bed-rest & Adequate Analgesia:
/ Liometacen inj.
Or: Indocid inj.
Or: FeldeneAmp.
Or: Voltaren Amp.
(1 inj-i.m. every 12-24 hrs)
Or: Indocid 25 mg Cap.
Or: Voltaren 25 mg Tab.
Or: Indomethacin 25 mg Cap.
Or: Brufen 400 mg Tab.
(1 cap. or 1 tab. after meals tid)
Or: Voltaren SR 100 mg Tab.
Or: Olfen SR 100 mg Tab.
Or: Indocid Retard 75 mg cap.
Or: Rheumafen SR 100 Cap.
Or: Feldene 20 mg Dispersible Tab.
Or: Feldene 20 mg Cap.
(1 tab. or 1 cap. daily)
2. Muscle Relaxants:
/ Coltramyl Amp.
Or: Norflex Amp.
(1 amp. i.m. every 12-24 hrs)
Or: Glifarelax Tab.
Or: Coltramyl Tab.
Or: Sirdalud Tab.
Or: Baclofen 10 mg Tab.
Or: Norgesic Tab.
Or: Norflex Tab.
Or: Myolgin Cap.
Or: Relax Cap.
(1 tab. or 1 cap. tid)
3. Physical Therapy Measures:
 Transuctaneous electrical stimulation of the painful area.
 Local application of heat
75


Massage & ultrasound
Traction to the pelvis or below the knees, either for a short period of
time during physiotherapy, or for hours with bed-rest.
 Injection for trigger points with a local anesthetic ± steroids e.g.
Depot-Medrol
 Active exercises as soon as the acute symptoms are relieved.
 Bracing in chronic or recurrent back pain.
B. Surgical Decompression:
Lumbar laminectomy & diskectomy are indicated in patients with loss of
bowel & / or bladder function, or progressive neurological deficit, or failure
of adequate conservative measures to relieve intractable pain.
CERVICAL SPONDYLOSIS & BRACHIALGIA
Neck & arm pain are frequently the result of degenerative changes of the
articulations of the cervical spine (spondylosis). Less commonly acute nerve
root compression occurs secondary to intervertebral disc herniation &
produces neck pain radiating into the arm with radicular sensory
impairment, muscle weakness, & deep tendon reflex abnormalities according
to the involved roots. Muscle spasm & straightening of the normal cervical
lordosis with limitation of neck movements are frequent. Plain x-rays, CT
scan-myelogram. Magnetic resonance imaging (MRI) of the cervical spine,
Pantopaque or Omnipaque myelo-graphy, & Electromyography (EMG)
confirm the diagnosis
Treatment:
A. Conservative Management:
 Neck immobilization utilizing a cervical collar which should be applied
with neck in a position of slight flexion.
 Cervical halter traction in cervical disc herniation.
 Appropriate analgesia; muscle relaxants; local heat therapy, local
injection, & other physiotherapeutic measures as mentioned in treatment
of low back pain & sciatica.
B. Surgical Decompression:
 This is indicated if the patient develops a neurologic deficit, or if pain
cannot be controlled by the conservative measures over a reasonable
period of time. Surgical decompression can be achieved through the
posterior approach (facetectomy & removing the compressing cartilage
or osteophytes) or by anterior decompression + anterior cervical fusion.
76

Vertebral artery & cervical sympathetic nerve irritation secondary to
osteophytic spur or soft disc herniation produce symptoms of
vertebrobasilar insufficiency from the resultant spasm (occipital pain,
headaches, tinnitus, vertigo, & drop attacks). Such patients are advised to
avoid the position that induces symptoms & to wear a soft cervical
collar periodically. If not .responding, surgical decompression of the
vertebral artery may be justified through an anterior-lateral approach.
 Brachial plexus irritation within the thoracic outlet secondary to spasm
of the scalene muscles: Cervical traction & collar are contraindicated as
they intensify the symptoms. Local heat, analgesics, & muscle relaxants
may help + supporting the arm in a sling.
 Cervical spondylosis frequently presents as a chronic syndrome with
periods of exacerbation (neck pain + referred pain to the trapezius &
deltoid region, the lateral aspect of the arm, the hand, the interscapular
area, & the occiput). Symptoms are reproduced by prolonged extension
of the cervical spine. Treated by:
/ Aspirin or Tylenol 1-2 tabs every 4 hrs.
‫ ساعات‬4 ‫ ْرص كع‬2-1
/ Coltramyl or Sirdalud tid.
 Home cervical traction; & Isometric cervical exercises will manage the
discomfortable exacerbations.
 Cervical spondylotic myelopathy needs surgical decompression of the
compromised cervical spinal cord ± bony stabilization.
 Extensive decompressive laminectomy is indicated in patients with
significant spinal canal stenosis whether congenital in origin or
secondary to multiple disc lesions & / or advanced spondylosis.
SEIZURE DISORDERS
THE EPILEPSIES
Epilepsy is a recurrent paroxysmal & transitory disturbance of the brain
function, which develops suddenly, ceases spontaneously, with or without
impairment of consciousness & abnormal electrical activity of the brain
(EEG changes)
CAUSES OF EPILEPSY:
I. Symptomatic Epilepsy:
A. Local Causes:
1. Congenital: e.g. congenital diplegia, epiloia & porencephaly.
2. Traumatic: e.g. birth trauma & head injuries.
3. Inflammatory: e.g. meningitis, encephalitis & neurosyphilis.
77
4. Vascular: e.g. cerebral atheroma, haemorrhage, thrombosis,
embolism & hypertensive encephalopathy.
5. Degenerative: e.g. presenile dementias.
6. Demyelinating e.g. diffuse sclerosis.
7. Space-occupying & increased ICT: e.g. brain tumours, brain abscess,
subdural haematoma & subarachnoid haemorrhage.
B. General Systemie Causes :
1. Toxic : e,g.
* Alcohol.
* Cacaine. Amphetamine.
* Chloroform.
* Amine-oxidase inhibitors.
* Camphor.
* Insulin.
* Metrazol.
* Imipramine.
* Strychnine.
* Barbiturates.
* Ether.
* Organophosphorous compounds.
2. Anoxic :e.g.
* Asphyxia.
* CO poisoning.
* Anaemia (severe).
* Nitrous oxide anaesthesia.
3. Metabolic : e.g.
* Hepatic coma.
* Porphyria.
* Hypoglycaemia.
* Pyridoxine (B) deficiency.
* Hypo-adrenalism.
* Uraemia.
* Hyperpyrexia.
* Alkalosis.
* Water intoxication.
* Parathyroid tetany
* Idiopathic hypoparathyroidism.
4. Endocrinal : e.g.
5. Nutritional: e.g.
78
* Pellagra.
* Beriberi.
* Kwashiorkor's disease.
* Rickets.
6. Allergic: e.g. in asthma & protein sensitization.
7. Febrile convulsions in children.
8. Iatrogenic: e.g.
* ECT (Electroconvulsive therapy).
* Metrazol.
* I.N.H.
* Cycloserine.
* Improper use of antiepileptics.
II. Idiopathic Epilepsy:
The cause is unknown.
CLASSIFICATION & CHARACTERISTICS
(as proposed by the ILAE)
I. Partial Seizures (Focal Seizures)
A. Simple partial seizures
1. With motor signs
2. With somatosensory or special sensory symptoms
3. With autonomic symptoms
4. With psychic symptoms
B. Complex partial seizures
1. Simple partial onset followed by impairment of consciousness
2. With impairment of consciousness at the onset
C. Partial seizures evolving to secondarily generalized seizures
1. Simple partial seizures
a) Evolving to generalized seizures
2. Complex partial seizures
b) Evolving to generalized seizures
3. Simple partial seizures evolving to complex partial seizures
evolving to generalized seizures
II. Generalized Seizures (Convulsive or Nonconvulsive)
A. Absence seizures
(1) Typical absence seizures (petit mal)
(2) Atypical
B. Myoclonic seizures
C. Clonic seizures
D. Tonic seizures
E. Tonic-clonic seizures (grand mal)
79
F. Atonic seizures
III. Unclassified Epileptic Seizures
Includes all those seizures that cannot be classified because of
incomplete or because they defy classification into the above categories;
for example, neonatal seizures with swimming movements.
IV. Status Epilepticus
PAROXYSMAL CONDITIONS RESEMBLING EPILEPSY
 Narcolepsy
 Migraine
 Paroxysmal abdominal pain (Abdominal epilepsy)
 Breath-holding Spells (Reflexive Apneic Spells)
 Hypercyanotic Attacks
 Shuddering Attacks
 Cardiovascular Syncope
 Conversion Reaction (Hysteria)
 Malingering
 Trigeminal Neuralgia (Tic douloureux)
 Paroxysmal vertigo
DRUG TREATMENT OF EPILEPSIES
Basic Principles & Guidelines for Antiepileptic Therapy
1. Baseline laboratory studies should be obtained prior to initiation of
antiepileptic therapy, e.g. complete blood count (CBC) & liver function
tests (LFT).
2. Serum blood levels of antiepileptics need to be followed & dosage
adjusted to achieve therapeutic range (see the table, vide infra).
3. Monotherapy is the best approach to newly diagnosed epileptics. The
drug of choice is selected according to seizure type (see the table, vida
infra).
4. Polypharmacy (multiple drugs) increases the risk of idiosyncratic side
effects & drug interaction
5. The dosage should be increased to achieve seizure control or to
maximum recommended dose, if seizures continue.
6. A second drug is considered only after failure to control seizures by the
first antiepileptic, or the occurrence of adverse side effects.
7. The "therapeutic range" should be used only as a guideline. Blood levels
of antiepileptics should be determined by the patient's side effects &
seizure control.
80
8.
Discontinuation of antiepileptic therapy can be tried in patients who
remain seizure-free for 2-3 years. It should be achieved gradually over
few months.
9. Recurrence of seizures has been reported in about one third of patients
after withdrawal of antiepileptics. They are usually controlled by reinstituting the same antiepileptic medications at the dosage regimen
prior to discontinuation.
10. Electroencephalography (EEG) or Brain Electrical Activity Mapping
(BEAM) is indicated in all types of epilepsies. It helps to identify seizure
type & plays a role in follow up of treatment. However, normal EEG
does not exclude the diagnosis of epilepsy.
11. CT scan of the brain, MRI &/or Cerebral Angiography are indicated in
selected cases, particularly in partial (focal) seizures, with or without
secondary generalization
ANTIEPILEPTIC DRUGS (AEDs)
Conventional AEDs
Drug
Indications
Phenobarbital
(Luminal, Sominal )
Phenytoin
(Epanutin )
Carbamazepine
(Tegretol)
Primidone
(Mysoline )
Ethosuximide
(Zarontin )
Methsuximide
(Celontin)
Valproic acid
(Depakine)
G.M.E., Partial
Seizures
G.M.E., Partial
Seizures
G.M.E., Partial
Seizures
G.M.E., Partial
Seizures
P.M.E.
Clonazepam
(Rivotril, Apetryl)
Diazepam
(Valium i.v.)
ACTH
Daily Dosage
Children
Adults
mg/kg
mg
3–5
60 – 200
Therapeutic
Bl. Level
mg/L
15 - 35
4–7
300 – 400
10 – 20
20 – 30
600 - 1200
6 – 10
10 – 25
750 – 1500
6 – 12
20 – 40
750 - 2000
50 – 100
P.M.E.
10 – 20
500- 1000
40 – 100
Partial
Seizures,P.M.E
G.M.E.
P.M.E.,
Myoclonus
Status
Epilepticus
Infantile
Spasms
30 – 40
1000-3000
50 - 100
0.01-0.2
1.5-20
0.01-0.07
0.15-0.2
10-150
40-60U/d
81
New AEDs
Drug
Indications
Starting
Dose
10 mg at
bedtime or 10
mg twice daily
Maintenance
Dose
20-30 mg/day
up to 60
mg/day
Plasma
HalfLife (hr)
30 – 46
Clobazam
(Frisium)
Partial &
generalized
seizures
Oxcarbazepine
(Trileptal)
Partial &
tonic-clonic
seizures
300 mg twice
daily
1200-2400
mg/day
8-24 for
active
metabolite
Tiagabine
(Gabitril)
Partial & 2ry
generalized
seizures
Not available
32-56 mg/day
6-8
Topiramate
(Topamax)
Partial & 2ry
generalized
seizures
100mg/day ↑
by100 mg/day
at weekly
intervals
400-1000
mg/day
20-24
Vigabatrin
(Sabril)
Partial & 2ry
generalized
seizures &
possibly infantile
spasms
500 mg twice
daily
Up to 3 g/day
4-8 (effect
lasts>3
days)
Zonisamide
(Excegran)
Partial & 2ry
generalized
seizures
100-200 mg/day
400-600
mg/day
Gabapentin
(Neurontin)
Partial seizures &
2ry generalized
seizures in adults
300 mg/day, ↑
by 300 mg/day
every 1-2 days
1200-2400
mg/day
50-68 (2738 with
enzymeinducing
drugs)
6
Lamotrigine
(Lamictal,
Lamotrine)
Partial seizures &
2ry
generalized
seizures in adults
25-50 mg
daily,↑ by 50
mg/day every 12 weeks
Up to 700
mg/day (100150 mg/day
with valproic
acid)
Felbamate
(Felbatol)
Partial
seizures &
secondarily
generalized
seizures in adults
400mg 2 or 3
times/day↑ by
400 to 600
mg/day every
2wks
1800-4800
mg/day
82
25 (12-14
with
enzymeinducing
drugs, 60
with VPA)
20-23
Seizures in
Children with the
Lennox-Gastaut
Syndrome (LGS)
15 mg/kg/d in 3
or 4 doses
(reduce
concomitant
phenytoin,
carbamazepine,
or valproic acid
by 20 to 33%)
with the dose ↑
by 15mg/kg/d
every 1-2 weeks
Up to 45
mg/day
Prophylactic Drug Treatment of Post-traumatic Seizures
Phenytoin & phenobarbital are recommended for patients who sustained
severe head injury. Post-traumatic seizures may occur early (occurring
whithin 7 days from the time of injury), or late (occur 1 or more weeks after
head trauma).
Phenytoin (Epanutin)
Loading Dose:
18 mg/kg, 25-50 mg/min. for 1hr.
Oral Maintenance Dose
5-7 mg/kg/day
Duration of Treatment:
6 months
Tapering (one drug at a time):
25% per week
Phenobarbital
1 mg/kg/hr for 4 hrs
1-3 mg/kg/day
6 months
25% per week
TONIC-CLONIC STATUS EPILEPTICUS
Two or more successive attacks of generalized convulsions without regaining
consciousness inbetween (one fit follow another without recovery of
consciousness). Also a prolonged tonic-clonic seizure lasting longer than 20
minutes is managed in the same way. Each seizure consists of loss of
consciousness + tonic-clonic convulsions + rolling of the-eyes + frothing +
tongue biting ± enuresis.
Treatment of Tonic-Clonic Status Epilepticus
Minutes
0-5


Action
Confirm diagnosis by observing seizure activity or one additional
seizure Oxygen by nasal cannula or mask; control head position &
airway; evaluate for intubation if ventilatory assistance needed
Obtain & record vital signs, continue to observe; treat any
abnormalities; establish ECG recording
83


6–9

10 –20

20+



>60





Obtain IV access, keep open with 0.9% saline; use glucometer or
draw venous blood for glucose, serum chemistries, hematology,
toxicology & AED levels
Evaluate oxygenation with oximetry or arterial blood gas
determination
If hypoglycemic, or if blood sugar measurement is not available,
give glucose: adults 100 mg thiamine IV followed by 50 ml 50%
glucose by IV push; children 2 ml/kg of 25% glucose
In adults, administer IV either 0.1 mg/kg lorazepam (Ativan) at 2
mg/min up to 4 mg total dose, or 0.2 mg/kg of diazepam (Valium)
at 5 mg/min up to 20 mg maximum
Diazepam can be repeated if seizures continue after 5 minutes
Diazepam must be followed by loading with phenytoin (Epanutin)
Load with 20 mg/kg phenytoin no faster than 50 mg/min in adults
& 1 mg/kg/min in children; monitor ECG and blood pressure
during infusion; IV fluids must be 0.9% saline. Fosphenytoin may
be given at 150 phenytoin equivalents/min as a safe substitute for
phenytoin
If status continues after 20 mg/kg phenytoin, give additional
phenytoin or fosphenytoin at 5 mg/kg until a maximum of 30
mg/kg
If status persists, give 20 mg/kg of phenobarbital IV at 60 mg/min
Expect apnea, particularly if the patient has received
benzodiazepines
Assist ventilation; intubation will be required
If status persists, use anesthesia with Pentobarbital (Nembutal),
midazolam (Dormicum), or proprofol (Diprivan), intubation,
ventilation & vasopressors will be required
Protocol for Refractory Status Epilepticus Treatment in Adults
 The patient must be managed in an ICU, intubated & placed on a
mechanical ventilator. Control of airway & ventilation will prevent
aspiration pneumonia. The patient must be monitored with an intensive
care protocol that includes recording BP, temperature, pulse &
respiratory function. Arterial access will allow assessment of blood
gases. Cardiovascular monitoring is facilitated by placement of a SwanGanz line. Strict input & output require bladder catheterization with a
Foley catheter
 Venous access is required, either a triple- lumen port or large-bore
peripheral IV for fluid bolus or dopamine administration
84









Midazolam (Dormicum): load with 0.2 mg/kg as a slow bolus &
maintain at 0.75-10 μg/kg/min
Propofol (Diprivan): initiate with 1 – 2 mg/kg & maintain at 2 – 10
mg/kg/hr
Pentobarbital (Nembutal): continuous EEG monitoring is required
during induction of the pentobarbital coma. Loading dose is 10 – 15
mg/kg IV at an infusion rate of 50 mg/min until a burst suppression
pattern or electrocerebral inactivity is observed on the EEG. Strive for 1
to 2 seconds of bursts & 5 to 10 seconds of suppression on the EEG.
Pentobarbital plasma level should be measured immediately after
administration of the initial or loading dose
The maintenance dose of pentobarbital is 0.5 – 1.0 mg/kg/hr
If hypotension develops, the rate of administration of the loading dose
can be decreased to 25 mg/min. This change in the maintenance dose can
be achieved by decreasing the dose to 0.5 mg/kg/hr. Hypotension is
managed with fluid bolus or dopamine to maintain a serum level of 10 –
20 mg/ml initially.
Maintain general anesthesia for an initial 12 hours, then assess a
minimum of every 24 hours if seizures continue
All other AEDs should be continued while the patient is initially
controlled with pentobarbital coma
Record an EEG daily
Continue to follow a schedule of anesthetic tapering & assessment
85
CEREBROVASCULAR DISEASES
BLOOD SUPPLY OF THE BRAIN
The brain receives its arterial blood from 2 systems:
1. The Internal Carotid Arteries (Rt. & Lt.): are branches of the common
carotids. They enter the skull through the carotid canals to the cavernous
sinuses.
Each internal carotid gives the following branches:
i. An ophthalmic artery.
ii. A posterior communicating artery.
iii. An anterior choroidal artery.
iv. An anterior cerebral artery.
v. A middle cerebral artery.
The Anterior Cerebral Artery: supplies the medial surface of the
anterior 3/5 of the cerebrum.
The Middle Cerebral Artery: supplies the lateral surface of the anterior
3/5 of the cerebrum.
2. The Vertebral Arteries (Rt. & Lt.): are branches of the Subclavian
arteries. Thsy enter the skull through the Foramen magnum. They unite at
the lower border of the pons to form the basilar artery which bifurcates at
the upper border of the pons into the 2 posterior cerebral arteries.
The Vertebro-Basilar System supplies the posterior 2/5 of the cerebrum, the
cerebellum, the brain stem & the spinal cord.
The Circle of Willis lies on the base of the brain (inferior surface) & is
formed by the anterior & posterior communicating arteries, anterior &
posterior cerebral arteries & the two internal carotids.
Blood Supply of the Internal Capsule:
i. Striate branches of the middle cerebral artery → the superior half of the
anterior limb, genu& posterior limb of the internal capsule.
ii. The recurrent artery of Heubner (a branch of the anterior cerebral
artery) → the inferior half of the anterior limb of the internal capsule.
iii. The posterior communicating artery → the anterior third of the inferior
half of the posterior limb of the internal capsule.
iv. Tne anterior choroidal artery (a. branch of the internal carotid) → the
posterior tow/third of the inferior half of the posterior limb of the internal
capsule.
SYNDROMES OF THE CEREBRAL ARTERIES:
Carotid Insufficiency: →Transient recurrent attacks of unilateral headache,
Ipsilateral blindness, contralateral hemiparesis or hemiparaesthesias
Convulsions, Confusion, Aphasia or dysphasia.
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Carotid Occlusion : → Ipsilateral blindness + Contralateral hemiplegia
cortical hemi-anesthesia & homonymous hemianopia ± Aphasia.
Middle Cerebral Artery Occlusion (Main artery):→Coma +Contralateral
hemiplegia, (marked in face, tongue & U.L.), Cortical hemi-anesthesia &
homonymous hemianopia ± Aphasia.
Middle Cerebral Occlusion (Striate branches): → Capsular Hemiplegia
(Complete hemiplegia + Subcortical hemi-anesthesia ± Hemianopia).
Anterior Cerebral Artery Occlusion (Main trunk): Contralateral hemiplegia (L.L. > U.L.) + Cortical anesthesia + Forced grasping & groping +
Motor aphasia, mental deterioration & apraxia on the left side (in leftsided lesion).
Heubner's Artery Occlusion (A branch of anterior cerebral): →
Contralateral facio-brachial monoplegia, proximal > distal + Spinothalamic sensory loss + Mental deterioration & motor aphasia in leftsided lesion.
Paracentral Artery Occlusion: → Contralateral crural monoplegia (L.L.) +
Cortical sensory loss -f- Urinary incontinence.
Posterior Cerebral Artery Occlusion (Main Trunk): → Contralateral
homonym mous hemianopia with macular sparing + Visual agnosia in
left-sided lesion + Temporal lobe seizures.
Thalamo-geniculate artery occlusion (A br. of posterior cerebral)
Thalamic syndrome.
Vertebro-Basilar Insufficiency : Brain stem dysfunction (Syncope +
Vertigo,. nausea, vomiting & deafness + Ophthalmoplegia + Ataxia +
Nystagmus + Bilateral pyramidal signs with bulbar palsy + Bilateral
dysae-sthesiae ± Migraine-like attacks or visual hallucinations).
Basilar Artery Occlusion : Deep coma + Small Fixed pupils + Pseudobulbar palsy & Quadriplegia + Decerebrate Rigidity. Usually Fatal.
Occlusion of the Paramedian Branches of the Basilar Artery:
Crossed Hemiplegias :
1. In the Midhraia ; → Weber's syndrome, Benedict's syndrome or Claude's
syndrome.
2. In the Pens : → Miltard'Gubler's syndrome or Foville's syndrome.
3. In the Medulla ObloDgata: Ipsilateral 9,10,11 & 12th cranial nerve
palsies & Hemi-ataxia-Contralateral Hemiplegia & Hemi-anesthesia.
Superior Cerebellar Artery Occlusion: → Ipsilateral cerebellar ataxia,
Homer's syndrome, deafness & choreiform movements + Contralateral
analgesia & thermo-anesthesia.
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Anterior Inferior Cerebellar Artery Occlusion : → Ipsllateral cerebellar
ataxia, Homer's syndrome, 5,7 & 8 th nerve palsies – 4 - Contralateral
analgesia & thermo-anesthesia.
Posterior Inferior Cerebellar Artery Occlusion (The Lateral Medullary
Syndrome of Wallenberg): Onset with vertigo, hiccup, vomiting,
dysphagia & facial pain + Jpsilateral cerebellar ataxia. Homer's
syndrome, 9 & 10 th nerve palsies & dissociated sensory loss over the
Face + Contralateral analgesia & thermo-anesthesia.
Spinal Artery Occlusion : Complete Flaccid paraplegia + Retention of urine
+ Sensory loss for pain & temperature below the level of the lesion with
preservation of touch & proprioceptive sensations (Posterior column).
CEREBRAL EMBOLISM
The Sources of Emboli:
1. The heart & great vessels:
i. Detached portion of a thrombus or a clot.
ii. Detached vegetations of bacterial endocarditis.
iii. Detached portion of the clol in myocardial infarction.
iv. Rheumatic heart disease, mitral stenosis & A.F.
v. Paradoxical embolism : e.g. femoral thrombosis or pelvic thrombophlebitis. Emboli may pass through a patent atrial septal defect & can
reach the brain.
2. The lung :
i . Thrombosis of a pulmonary vein.
ii. Bronchogenic carcinoma.
iii. Lung abscess or bronchiectasis.
3. Other sources of emboli :
i. Fat embolism.
ii. Air embolism.
iii. Tumour embolism.
iv. Parasitic emboli.
CEREBRAL THROMBOSIS
Aetiology : It may occur secondary to :
1. Disease of vessel wall :
i. i . Atheroma.
ii. Syphilitic endarteritis.
iii. Polyarteritis nodosa.
iv. Thromboangiitis obliterans.
v. Congenital aneurysm of Circle of Willis.
2. Diseases of the blood :
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i . Polycythemia vera.
ii. Severe anaemia.
3. Circulatory Disorders :
i. Hypotension & shock.
ii. Myocardial infarction..
CEREBRAL HAEMORRHAGE
Aetiology :
1. Arteriosclerosis.
2. Hypertension.
3. Other causes :
* Angiomatous malformation.
* Acute encephalitis.
* Anaphylaxis.
* Purpura
* Whooping cough.
* Anaemia.
* Leukaemia.
* Septicaemia & Pyaemia.
* Haemorrhage into a brain tumour.
Clinical Picture :
1. Age : Late middle life.
2. Sex : Males more than females.
3. Onset : Sudden, abrupt or apoplectic.
4. Premonitory symptoms :
Headache, Vamiting, Dizziness, Vertigo, Syncope, Confusion, Epistaxis,
Transient monoparesis &/or dysphasia.
5. Presenting Symptoms :
i. Headache.
ii. Vomiting.
iii. Coma.
iv. Convulsions.
v. Focal signs according to the site & size of haemorrhage.
CEREBRAL ARTERIOSCLEROSIS
Clinical Picture :
1. Onset : is insidious.
2. Course : is slowly progressive.
3. Mental symptoms :
* Amnesia for recent events ± confabulation.
* Emotional instability.
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* Delusions.
* Depression.
* Confusion.
* Dementia.
4. Epileptic/its.
5. Aphasia, agnosia & apraxia.
6. Pyramidal signs : monoplegia, hemiplegia, double stroke, ... etc.
7. Extrapyramidol signs: Parkinsonism, hemiballismus, senile tremor &
athetosis.
8. Arteriosclerotic retinopathy.
9. General arteriosclerosis.
10. Hypertension.
SUBARACHNOID HAEMORRHAGE
Causes:
1. Ruptured I.C. aneurysm (85%).
2. Ruptured I.C. angioma (10%).
3. Other causes :
* Head injury.
* Haemorrhagic diseases.
* Anticoagulants.
* Acute encephalitis.
* I.C. tumours.
* Cerebral atheroma with hypertension.
* Angioma of the cord.
* Cheese syndrome as a compleication of MAOI.
Clinical Picture:
1. Increased ICT. :
* Headache, Vomiting, Papilloedema.
* Convulsions, Coma or confusion.
* Retinal haemorrhages.
* Extensor plantar response, Hyporeflexia, lost abdominals.
* Hyperpyrexia, transient hypertension, albuminuria & glycosuria.
2. Meningism (Meningeal irritation):
* Neck rigidity.
* Positive Kernig's sign.
* Opisthotonos.
* Low backache & pain in the back of neck.
3. Focal Symptoms:
* Visual field defect.
90
* 3 rd, 4th. & 6 th. nerve palsies.
* Mental symptoms, hemiparesis± motor aphasia (in frontal lobe haemorrhage).
* Convulsions & monoplegia (middle cerebral).
* Crossed homonymous hemianopia (Posterior cerebral).
* Quadriplegia or crossed hemiplegia (basilar artery).
* Low back pain & pain in L.Ls., Sphincteric disturbances, stiffness of the
spine, Kernig's sign, flaccid weakness of L.L.s. with anaesthesia &
areflexia (Spinal subarachnoid haemorrhage);
4. Cranial Signs :
* Cranial bruit.
* Angiomaoftheface.
* Pulsating exophthalmos.
5. CSF:
* RBCs. in the first, week.
* Xanthochromia for 2-3 weeks.
* Raised protein content.
* Mononuclear pleocytosis.
* ± Paretic colloidal gold curve.
6. X-ray skull may show:
* Calcified angioma.
* Calcified wall of an aneurysm.
* Abnormal vascularity.
* Erosion of bone.
7. Angiography : Bilateral carotid angiography should be done except in very
old, severely shocked, hypertensive, atherosclerotic patients.
Prognosis:
i. 40—50% die within 8 weeks, 2/3 of them die within 24 hours.
ii. 20 % die within 6 months due to recurrent bbeding. UL 10 % are symptomfree, 10% are disabled by hemiplegia, epilepsy, etc. & 10 % have minimal
residual symptoms.
Treatment :
\
A. Medical :
* Complete bed rest for 4 weeks at least.
* Morphine or pethidine.
* Antibiotics.
* Avoid straining & exertion & open bowels.
* Dehydrating measures.
* Lumbar puncture.
91
* Management of coma as usual.
B. Surgical:
* Carotid ligation.
* Excision of the aneurysmal sac or application of a surgical clip to its neck.
TRANSIENT ISCHEMIC ATTACKS (TIAS)
T.I.As. are short-lived attacks of cerebral ischaemia, each one lasts for few
minutes to few hours (maximally 24 hours), commonly produced by emboli
arising from atheromatous plaques in the extracranial portions of the carotid
or vertebral arteries, or the large intracranial arteries, or from clots formed
in the heart, or caused by a fall in arterial perfusion pressure. Clinically,
T.I.As. either present with signs & symptoms of carotid insufficiency (e.g.
transient blindness of one eye & hemiparesis ± hemi-hypoesthesia in the
opposite U. & L.Ls.); or with signs of vertebro-basilar insufficiency (e.g.
dizziness, vertigo, unsteadiness, diplopia, impairment of consciousness... etc).
Patients with recurrent T.I.As. have a higher risk of developing a stroke than
the rest of the population.
Carotid artery TIAs:
Diagnostic criteria:
 Contralateral weakness or numbness in about 50% of patients
 Dysphasia, apraxia, &/or confusion if the dominant hemisphere is
involved
 Transient blurring of vision or blindness in ipsilateral eye (amaurosis
fugax) in about 40% of patients
 Ipsilateral headache of vascular type Aids for bedside diagnosis
 Abnormal pulsation of the external carotid branches or common carotid
arteries on either side
 Arterial bruit localized over either carotid bifurcation &/or the contralateral orbit.
 Hemorrhages, microemboli, & exudates confined to the ipsilateral retina.
 Diminished pressure in the ipsilateral ophthalmic artery.
 Diminished temperature over the medial aspect of the ipsilateral
forehead
Vertebral basilar TIAs:
Diagnostic criteria:
1. Upper spinal cord & lower brainstem (ant. spinal & vertebral arteries)
a. Weakness or paralysis of legs or all four extremities (drop attacks)
with preservation of consciousness
92
b.
Weakness & vertigo precipitated by rotation or extension of the
head
c. Ataxia
d. Dysarthria & dysphagia
e. Unilateral or bilateral numbness
f. Occipital headache
2. Labyrinth & cochlea (internal auditory artery)
a. Vertigo, nausea, & vomiting
b. Tinnitus
c. Acute onset of deafness
3. Pons & midbrain (basilar artery)
a. Occipital headache
b. Lightheadedness &/or syncope
c. Confusion or coma
d. Diplopia
e. Unilateral or bilateral numbness or weakness
4. Cerebral hemispheres, occipital lobes, & temporoparietal areas (posterior
cerebral artery)
a. Homonymous visual field loss
b. Blindness, cortical type
c. Temporal lobe seizures
Aids for bedside diagnosis
 Bruits at the subclavian-vertebral junction & along the course of the
vertebral artery to the mastoid
 Unequal blood pressures in the two arms (subclavian steal syndrome)
 Brachial arm blood pressures reduced bilaterally in relation to ophthalmic artery pressures (bilateral subclavian steal)
N.B.: Vascular pathology is assessed by cerebral angiography.
However, not all patients with TIAs are candidates for angiography
Investigations of TIA Patients:
1. Hematological screening: Platelet count & hematocrit.
2. Cardiac evaluation: ECG, Echocardiography, & Holter monitoring.
3. Noninvasive Tests:
 Measuring flow in tributaries:
 Directional Doppler
 Oculoplethysmography
 Ophthalmodynamometry
 Radionuclide angiography
 Thermography Direct bifurcation data:
93
 Carotid phonoangiography
 Real-time echo
 B-mode ultrasound (duplex scanning)
4. Angiography:
 Standard cerebral angiography
 Digital subtraction angiography (DSA)
¤ Digital venous
¤ Digital arterial
5. Other Cerebral Blood Flow Studies:
 Positron emission tomography (PET)
 Single photon emission computed tomography (SPECT)
Treatment:
1. Controlling risk factors e.g. hypertension, diabetes mellitus, IHD,
hyperlipidemia, obesity, smoking, lack of adequate exercise.
2. Specific therapy of the vascular pathology causing the T.I.As.:
a. Antiplatelet agents e.g. Aspirin, Persantin, Ticlid, Plavix
b. Anticoagulants e.g. Heparin, Warfarin (Marevan)
c. Endarterectomy
d. External carotid branch to middle cerebral or vertebrobasilar branch
shunts
e. Improving cerebral perfusion e.g. correction of hypotension, treating CHF, avoiding head elevation, keeping the patient in bed, adequate hydration.
f. Antiarrhythmic drugs for arrhythmia
g. Cardiac surgery for ventricular aneurysm, valvular disease &
myxoma.
Treatment Guidelines in T.I.As.
Location of Lesion
Severity
1.
Penetrating vessel (Lacunar disease)
2.
Extracranial carotid artery
disease (internal carotid
bifurcation)
Treatment
Control of hypertension
Antiplatelet agents
Nonstenosing plaque
Endarterectomy (Warfarin
Severe stenosis (less
if no surgery)
than 2 mm lumen)
Bed-rest + heparin for 1-2
Occlusion
wks.
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3.
Anterior circulation largevessel disease (Internal
carotid siphon or Middle
cerebral main stem)
4.
Posterior circulation occlusive disease
Plaque
Tight stenosis
Occlusion
Antiplatelet agents
Warfarin (Marevan)
Consider ECA-MCA
bypass

Vertebral artery (neck) Plaque or Stenosis
Occlusion

Vertebral artery (1C)
Plaque
Stenosis
Antiplatelet agents
Warfarin (Marevan)

Bilateral vertebral
artery (1C)
Occlusion or Severe
stenosis
Consider occipital PICA
shunt

Basilar artery
Plaque
Stenosis
Occlusion
IC= Intracranial
ECA = External carotid artery
Antiplatelet agents
Short-term heparin
Antiplatelet agents
Warfarin (Marevan)
Short-term heparin
MCA = Middle cerebral artery
PICA = Posterior inferior cerebellar artery
Antiplatelet Therapy of TIAs:
/ Baby Aspirin Tab., 75 mg
Or: Aspocid Inf. Tab. 75 mg
(1 tab daily for 12 months. Discontinue gradually. Repeat if TIAs recur)
/ Persantin 75 mg Tab. (1 tab before meals tid- qid for 1 yr. Discontinue
gradually. Repeat if TIAs recur)
Or: Persantin-Plus Cap. (1 cap, a.c., tid)
Or: Plavix 75 mg Tab. (1 tab o.d.)
Or: Ticlid 250 mg Tab. (1 tab bid)
Anticoagulation Therapy of TIAs:
/ Heparin 5000 U Amp.
(5000-10000 units i.v. bolus initially, then i.v. infusion of heparin in 5%
dextrose at a rate of 1000-1500 U/hr for 7-10 days at least)
N.B.: The aim is to maintain the activated partial thromboplastin time (a
PTT) at 2 to 2.5 times the control value.
/ Marevan Tab., 5 mg
Or: Dindevan Tab., 50 mg
(2-3 tabs, daily starting on the 3rd-5th day of heparin therapy & continuing for 3-5 days, then adjust the dose according to PT. Continue therapy for
3 months after satisfactory control of symptoms, then discontinue gradually
over several weeks. To be repeated if TIAs recur again)
95
N.B.: The aim is to keep the prothrombin time (PT) at 1.5 to twice the control
value, or to keep the INR between 1.5 – 2.5
96
REVERSIBLE ISCHEMIC NEUROLOGICAL DEFICIT (RIND)
STROKE - IN - EVOLUTION, & COMPLETED STROKE

Episodes of neurologic dysfunction due to vascular disease which
resolve in less than 24 hrs are known as TIAs.
 If the neurologic deficit resolves completely within 3 weeks, it is called
reversible ischemic neurological deficit (RIND).
 A completed stroke (an established brain infarction) is one in which the
neurologic deficit becomes static & there have been no new events
within the preceding 24 hrs.
 An evolving or progressing infarction (stroke-in-evolution) is one in
which new signs & symptoms develop during the preceding 24 hrs.
Treatment of TIAs, RIND, & Stroke-in-Evolution is the same
ACUTE STROKES
Neurologists used to be very much enthusiastic to diagnose the
underlying vascular pathology of strokes by clinical methods alone.
 After the introduction of CT brain scanning, it has been realized that
such clinical confidence may prove incorrect. In many instances, a
clinical diagnosis of an ischemic stroke proved to be hemorrhagic by CT
scanning, or a hemorrhagic one proved to be an infarction.
 Strokes may be caused by: cerebral hemorrhage, cerebral embolism, or
non-embolic infarction.
 Uncommon causes of strokes include: arteritis, polycythemia, syphilis,
Ml, acute GIT hemorrhage, infective endocarditis (low-grade fever,
cardiac murmur + positive blood culture), ruptured cerebral aneurysm
or AVM.
Investigations include:
 CT brain scanning: differentiates cerebral infarction, hemorrhage,
abscess, & tumor. However, it may be negative in some ischemic strokes,
MRI brain is most helpful in these cases.
 Lumbar puncture & CSF examination may help differentiating cerebral
hemorrhage from infarction. However it is contraindicated in gross
papilledema.
 Other investigations e.g. CBC, ESR, RBS, VDRL, ECG, chest X-ray &
skull X-ray.
The following conditions may mimic strokes:
 Cerebral tumours, primary & metastatic
 Subdural hematoma.

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
Cerebral trauma. However, head injury may be caused by a fall as a
result of the stroke.
 Hypoglycemic episodes.
Management of Strokes:
1. Care of the comatose patient:
 Ensure a clear airway
 Semi-prone position
 Attention to the state of hydration.
 Care of the skin.
 Care of the bladder & rectum.
 Chest physiotherapy.
 Regular passive exercises of the paralysed limbs.
 Positioning of the paralysed limbs in the optimal positions.
2. Thrombolytic Therapy: e.g. recombinant tissue plasminogen activator
(rTPA). This is recommended in patients with acute ischemic stroke
(confirmed by CT scan), within 3 hours from onset of symptoms
(Therapeutic time window), in those fulfilling the inclusion criteria &
who have no exclusion criteria.
/ Actilyse inj. (0.9 mg/kg, max. 90 mg, 10% of the dose is given as an i.v.
bolus and the remainder is given by i.v. infusion over 60 min.)
3. Anticoagulant Therapy is indicated in the following situations:
 During the acute phase of cerebral embolism & also as prophylaxis
against further embolic episodes.
 To limit the progression of "Stroke-in-evolution"
 TIAs.
/ Heparin Injection (5000 U / ml) (1,000 units/hr i.v. infusion over 24 hrs
for 36-48 hrs).
/ Marevan Tablets.
(5 mg tid for 1 day; 5 mg bid for 1 day; then 5 mg daily. Adjust the dose
according on PT, PC, & INR)
N.B.: Anticoagulants are valueless in a fully developed stroke as a result of
non-embolic infarction.
4. Hypotensive therapy:
 Oral hypotensive drugs are given to patients known to be
hypertensives.
 Recently discovered hypertension should be managed cautiously.
5. Surgery is indicated in the following situations:
 An expanding intracerebral hematoma.
 Spontaneous cerebellar hemorrhage or massive infarction.
98

Surgery of the great vessels (carotid or vertebral arteries) is
indicated in TIAs attributed to emboli arising from ulcerated
atheromatous plaques of such vessels, or to significant stenosis.
CARDIOGENIC EMBOLIC STROKES

Over 80% of cerebral infarctions are due to arterial thrombosis or arteryto-artery embolism.
 About one sixth of brain infarcts are due to emboli of cardiac origin.
 Atrial emboli occur in rheumatic heart disease (RHD), mitral stenosis &
atrial fibrillation (AF).
 Ventricular emboli occur in acute myocardial infarction (MI), post-MI
ventricular aneurysm, & cardiomyopathy.
 Valvular emboli occur in infective endocarditis, prosthetic heart valves &
mitral valve prolapse (MVP)
Treatment:
1. Prophylactic anticoagulation is indicated in all patients at risk of systemic embolization.
2. All patients with cardiogenic embolic stroke should be heparinized
immediately unless they have large infarct, hypertension, or CT scan
evidence of hemorrhagic infarct, or if anticoagulants are contraindicated.
3. Aneurysmectomy for stroke prevention is indicated in selected cases.
HYPERTENSIVE ENCEPHALOPATHY

It may complicate essential hypertension, toxemia of pregnancy,
posterior fossa mass lesions, & ischemic processes.
 It presents with impairment of consciousness, focal neurologic deficits,
seizures, & elevated ICP.
Management:
1. Frequent assessment of arterial BP (every 1-2 min) by automated
sphygmomanometry.
2. The mean arterial pressure (MAP) should be lowered to the high end of
the patient's premorbid BP range, or to (130 ± 10 mm Hg). Avoid vigorous lowering of BP. MAP = 1/2 (systolic BP) + 2/3 (diastolic BP).
3. The drug of choice is IV sodium nitroprusside (Nipride). S.L. nifedipine
(Adalat, Epilat) is also very useful.
/ Nipride 50 mg Vial + 500 ml D5/W (100 ug/ml)
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(3 ug/kg/min, IV by infusion pump, better via a central line, until the desired BP reduction is obtained)
Or: Adalat 10 mg Cap.
Or: Epilat 10 mg Cap. (10 mg S.L. PRN)
N.B.: Reflex sinus tachycardia is controlled by:
/ Inderal Amp., 1 mg or 5 mg (1-5 mg i.v. PRN)
4. Alternative drugs include: diazoxide (Hyperstat), Hydralazine
(Apresoline), & labetalol (Trandate):
/ Hyperstat 300 mg Vial (150 mg i.v. bolus, undiluted, over 30 sec).
Or: Apresoline 20 mg Amp., (IV. infusion of 10-20 mg/hr)
Or: Trandate 100 mg Amp., (IV. infusion of 20-160 mg/hr)
N.B.: Oral antihypertensive agents should be started immediately to maintain
adequate BP control after tapering & D/C of i.v. agents.
5. Posterior fossa mass lesions complicated by hypertension should be
treated by: (a) Lowering ICP e.g. by dehydration therapy (IV Mannitol
20%, Decadron, & Lasix), & intubation & hyperventilation
(b) Controlling BP (c) Surgical decompression when indicated.
6. Toxemia of pregnancy (Hypertension, seizures, proteinuria, edema,
confusion, focal neurological deficits):
 Control BP by i.v. infusion of Nipride
 Control seizures by anticonvulsants
 Cesarean section.
SUBARACHNOID HEMORRHAGE (SAH)
Causes Include:
1. Ruptured intracranial "berry" aneurysm.
2. Ruptured intracranial AVM or angioma.
3. Arterial rupture in hypertension & atherosclerosis.
4. Less common causes e.g. rupture of a mycotic or atheromatous
aneurysm, bleeding from an intracranial tumor, blood dyscrasias,
anticoagulant therapy, head injury, or intraventricular leakage of an
intracerebral hemorrhage.
Symptoms include:
1. Severe headache, initially occipital in location.
2. Vomiting, photophobia, & discomfort on bending the neck.
3. Loss of consciousness.
Signs include:
1. Patient appears distressed, confused, & disoriented + vomiting.
2. Neck stiffness.
100
Kernig's sign (Attempting to extend the knee with the hip flexed at 90°
→ spasm of the hamstring muscles)
4. Focal neurological deficits e.g. ophthalmoplegia.
5. Subhyaloid hemorrhages & papilledema.
6. Other signs e.g. a temporary rise of BP, pyrexia. & transient glycosuria.
 SAH should be differentiated from meningitis.
 CSF is under increased pressure, bloody, xanthochromic supernatent
within 24 hrs. & increased protein content.
 Lumbar puncture is avoided in presence of papilledema or lateralizing
signs.
 CT brain scanning is diagnostic Past history of strictly unilateral
migraine, seizures, previous attacks of SAH, & presence of audible
cranial bruit favour the diagnosis of AVM.
 ECG discloses evidence of hypertension in hypertensive patients & IHD
(MI) in some cases.
 MRA in helpful & cerebral arteriography prior to considering surgery.
Treatment:
 Ensure a clear airway:
 Nursing care of comatose patient.
 Bed-rest for at least 4 weeks, then allow moving out of bed for
increasing periods, then convalescence for one month.
 Hypotensive therapy to control blood pressure.
 Surgery to prevent rebleeding which could be fatal. & for removal of an
expanding hematoma.
 Treat headache, vomiting, & confusion by:
/ Pethidine inj. (100 mg i.m., repeated as necessary)
/ Neurazine Amp. (50 mg i.m., repeated as necessary)
 Antifibrinolytic therapy:
/ Epsikapron Tabs. & inj. (Epsilon-aminocaproic acid)
(36 gm/day orally or by NGT or i. v.)
Or: Cyclokapron Tabs. & inj. (Tranexamic add)
(6 gm/ day orally or by NGT or i.v.)
 Calcium Antagonist (Nimodipine) for prevention & treatment of ischemic
neurological deficit caused by cerebral vasospasm following S.A.H.
/ Nimotop 10 mg Bottles (50 ml)
(1 mg-5ml Nimotop infusion/hr. for 2 hrs., if no marked fall in B.P. give 2
mg-10 ml Nimotop infusion fluid/hr. Patients weighing < 70 kg or with
labile B.P. are given half the above mentioned doses. Treatment should
be continued up to the 10th to 14th days after S.A.H.)
3.
101
/ Nimotop 30 mg Tablets.
(2 tabs 6 hourly for 7 days following the period of i.v. infusion)
INTRACEREBRAL HEMORRHAGE

Cerebral hemorrhage has a better prognosis than cerebral infarction,
because far less tissue may be destroyed.
 Causes of cerebral hemorrhage include hypertension, arteriovenous
malformations (AVM), coagulopathies, anticoagulation & antiplatelet
therapy.
Management:
A. Surgery is indicated when intracranial pressure (ICP) is high or the
patient's condition is deteriorating. Evacuation of the hematoma &
treating the cause e.g. AVM.
B. Medical Treatment:
1. Maintain adequate airway, with endotracheal intubation if necessary
2. Control hypertension:
/ Epilat-Retard 20 mg Tab. (1 tab. daily)
/ Capoten 25 mg Tab. (1 tab bid or tid)
3. Dehydration therapy to reduce ICP:
/ Mannitol 20% Solution (50-100 gm i.v. over 20 min).
/ Decadron Injection (32 mg i.v.)
+ Hyperventilation
4. Correct fluid & electrolyte disturbance
5. Anticonvulsants e.g.:
/ Epanutin 250 mg Amp. (Slowly i.v. every 12-24 hrs.)
Superficial Supratentorial Hemorrhages:
 Conservative management is indicated if no signs of increased ICP or
progressive deterioration.
 Surgical evacuation is indicated if the level of consciousness is
deteriorating or signs of increased ICP occur.
Thalamic & Putamen Hemorrhages:
 The prognosis is almost always poor, particularly in comatose patients
with large hemorrhages, regardless of treatment.
 Operation can be lifesaving in patients with small lesions who show
progressive deterioration.
Pontine Hemorrhages:
 Rapid coma + pinpoint pupils + quadriparesis. Recovery is rare.
Surgery is hazardous
102
 Conservative management is the role in most cases.
Cerebellar Hemorrhage:
 Hypertension + severe headache + ataxic gait + brainstem dysfunction
e.g. paralysis of ipsilateral gaze.
 Early decompression of the hemorrhage is mandatory when the lesion is
greater than 2-3 cm even in comatose patients. Biopsies of the lesion
wall may disclose an occult AVM, hemangioblastoma, or other tumors.
Intraventricular Hemorrhage:
 Causes include a ruptured intracranial aneurysm, AVM, hypertension,
tumor,... etc.
 Immediate drainage of the ventricle may be necessary to reduce high
ICP.
 The prognosis is grave when a cerebral hemorrhage ruptures into the
ventricles.
 Removal of an intraventricular AVM or a benign tumor can be very
rewarding.
Hemorrhage from Aneurysms, & AVMs:
 Removal of the hemorrhage & excision of the causative lesion are performed either in one operation or in two steps.
VASCULITIS OF THE CNS
Primary Vasculitides:
 Polyarteritis nodosa
 Temporal arteritis
 Takayasu's arteritis
 Isolated angiitis of the CNS
 Behcet's disease
Secondary Vasculitis:
 Infection e.g. bacterial, fungal. & viral infections
 Toxins e.g. abuse of amphetamines.
 Collagen vascular disease e.g. rheumatoid arthritis, scleroderma, SLE
 Neoplasms e.g. Hodgkin's disease, other lymphomas, carcinoma.
leukemia, & multiple myeloma.
Systemic Inflammatory Diseases Associated with CNS Vasculopathies:
 Systemic lupus erythematosus (SLE)
 Thrombotic. thrombocytopenic purpura.
103
N.B.: Confirmation of the diagnosis depends on cerebral angiography &
brain biopsy. Other lab. studies include CBC. ESR, Ra latex (R.F.)
Treatment:
1. Removing the offending agent, in the secondary vasculitides. e.g.,
antibiotics for infection, discontinuation of drug or toxin, treatment of
neoplasms & Corticosteroids to suppress inflammation.
/ Hostacortin 5 mg Tab.
(40 mg/day for 1 week, 20 mg/day for 1 wk, & 10 mg/day for 1 wk).
2. Corticosteroids are the major therapeutic agents in SLE, Thrombotic,
thrombocytopenic purpura, Temporal arteritis. & Isolated angiitis of the
CNS
/ Hostacortin 5 mg Tab.
Or: Hostacortin-H 5 mg Tab.
(60 mg/day initially, to be gradually reduced to a maintenance dose
sufficient to control symptoms. Treatment usually continues for 6 months
to 1 yr. Gradual discontinuation over few months is needed).
N.B.: Antacids (Mucaine or Epicogel or Zantac), Calcium; Addi-K or Slow-K
are needed.
3. Cyclophosphamide (Endoxan) or Azathioprine (Imuran):
/ Endoxan 50 mg Tab.
(75-150 mg daily A.M., Check CBC & urinalysis for RBCs)
Or: Imuran 50 mg Tab.
(50-150 mg daily, Check CBC
4. Other Treatments:
 Indomethacin (Indocid)
 Colchicine (Colmediten)
 Prostaglandins
 Chlorambucil (Leukeran 2 mg or 5 mg Tab.)
 Methotrexate (Methotrexate 2.5 mg Tab.)
 Cyclosporin (Sandimmun, Neoral)
104
CNS INFECTIONS
ACUTE MENINGITIS
Establishing the Diagnosis:
 Fever, vomiting, headache, photophobia, neck rigidity, altered mental
status, & seizures.
 CT scan should precede lumbar puncture if there are focal seizures. or
neurologic deficit suggesting a brain abscess or other S.O.L. Lumbar
puncture & CSF examination: show increased tension, cells
(polymorphonuclear) & protein content, with diminished sugar content
(consumed by bacteria).
 Blood & CSF cultures & sensitivity tests are needed. The commonest
causative organisms are: E coli & Group B strepto-cocci in infants
below 8 weeks of age; H. influenzae in children; Meningococci in young
adults; Pneumococci in the elderly.
Treatment:
A. Antibiotic Therapy:
(1) Initial Antibiotic Therapy:
(Until the result of CSF culture is available)
Initial Therapy of Bacterial Meningitis
Clinical situation
Neonates
Drug of choice
Ampicillin & gentamicin
Or Ampicillin & ceftriaxone
Alternative
Vancomycin &
gentamicin
Infants & children
Ampicillin & chloramphenicol or ceftriaxone
Erythromycin &
chloramphenical
Adults
Ampicillin & ceftriaxone
Erthromycin &
chloramphenicol
Neurosurgical
infection
Vancomycin & cefrazidime
Vancomycin &
gentamicin
Basilar skull fr.
or CSF leak
Vancomycin & ceftazidime
Erythromycin &
chloramphenical
Immunosuppression
or malignancy
Ampicillin & ceftazidime
Erythromycin
(or vancomycin)
& gentamicin
105
(2) Modification of Antibiotic Therapy:
(On the basis of culture & sensitivity testing)
Antibiotic Therapy of Bacterial Meningitis of Known Etiology:
Alternative (for pts.
allergic to penicillin)
Organism
Drug of choice
Gram-positive organisms
Streptococcus
pneumoniae
(pneumococcus)
Ceftriaxone plus
vancomycin
Ceftriaxone plus
rifampin
Erythromycin
Penicillin G
Erythromycin
Listeria monocytogenes
Penicillin &
gentamicin
Oxacillin or
nafcillin
Ampicillin
Vancomycin &
gentamicin
Vancomyicn
Penicillin G
Trimethoprimsulfamethoxazole
Chloramphenicol
Gram-negative organisms
Meningococcus
Penicillin G
Third-generation
cephalosporin
Chloramphenicol
Chloramphenicol
Streptococcus, groups A
&B
Streptococcus, group D
(enterococcus)
Staphylococcus
Haemophilus influenzae
Enteric gram-negative
rods
(Escherichia coli, proteus
species, Klebsiella
species)
Pseudomonas aeruginosa



Ampicillin or
third-generation
cephalosporin
Third-generation
cephalosporin or
ticarcillin plus
gentamicin
Ticarcillin (or
ceftazidime) +
gentamicin
Optional
intrathecal
drug
Gentamicin
Bacitracin
Gentamicin IV & IT
Gentamicin
Gentamicin IV & IT
Gentamicin
Repeated L.P. every 24-48 hrs to monitor response to therapy as
indicated by CSF sterilization & normalization.
Antibiotic therapy should continue for 10 to 14 days & the patient should
be afebrile for at least 5 days before its discontinuation. & the CSF
glucose should return to normal.
Staphylococcal meningitis needs therapy for 4 to 6 weeks, as
endocarditis & other serious systemic infections are frequent.
106

Antibiotics are administered IV except for gentamicin (Garamycin),
amikacin (Armikin), & tobramycin (Nebcin) which are given IM.
Penicillin G:
 Neonates (up to 2 months): 150,000 - 200,000 U/kg/day IV (q6/ 8 hrs)
 Children < 50 kg: 250.000 U/kg/day IV (every 4 hrs)
 Adults: 20,000,000 U/day IV (every 4 hrs)
Ampicillin:
 Neonates up to 1 wk: 100mg/kgf/day (every 6 hrs)
 Neonates 1 wk-2 months: 150-200 mg/kg/day i.v. (every 6 hrs)
 Children 50 kg: 200-400 mg/kg/day i.v. (every 4 hrs)
 Adults: 4 gm/day IV (every 4 hrs)
Chlorampnenicol (Cidocetine, Miphenicol, Kemlcetine):
 Premature & Full-term in 1st week: 25 mg/kg/day IV (every 12 hrs)
 Full-term infants In 1st month: 50mg/kg/day IV (every 8 hrs)
 Full-term infants 1-2 months: 50-100 mg/kg /day iv (every 6 hrs)
 Children 50 kg: 75-100 mg/kg day IV (every 6 hrs)
 Adults: 3-4 gm/day i.y. (every 4 hrs}
Cefotaxime (Claforan):
 Neonates 2 months: 100 mg/kg/day IV (every 8 hrs)
 Children 50 kg: 150 mg/kg/day IV (every 6 hrs}
 Adults: 6 gm/day IV (every 8 hrs)
Gentamicin (Garamycin), Tobramycin (Nebcin):
 Neonates in first week: 5 mg/kg/day IM or IV (every 12 hrs)
 Neonates 1 wk-2 months: 7.5 mg/kg/day IM or IV (every 8 hrs)
 Children 50 kg: 4 mg/kg/day IM or IV (every 8 hrs)
 Adults: 3-5 mg/kg/day IM or IV (every 8 hrs) + 4-5 mg intrathecal or
intraventricular every 2 days.
Amikacin (Amikin):
 Neonates in first week: 10 mg/kg loading dose, then 7.5 mg/kg/day IV
(every 12 hrs)
 Children & Adults: 75 mg/kg/day IV (every 8 hrs) + 4-5 mg intrathecal
or intraventricular in adults or shunt infections.
Metronidazole (Flagyl): in Bacteroides species
(15 mg/kg IV over 1 hr Then 30 mg/kg/day IV (every 6 hrs)
B. Supportive Therapy:
(1) Dehydration Therapy (for cerebral edema, papilledema & coma)
 Hyperventilation to a PCO2 of 28 torr
/ Mannitol 20% Solution (100gm i.v. over15-20 min)
107
/ Decadron Vial (8 mg i.v. every 6 hrs)
(2) Septicemic shock may call for:
 Volume replacement (For hypovolemia)
/ Intropin Amp.. 200 mg/5 ml, (IV infusion for hypotension)
/ Solu-Medrol Vial, (30 mg/kg IV)
(3) Disseminated intravascular coagulation (DIC):( platelets & fibrinogen
levels + Prolonged PT & PTT + Fibrin degradation products in blood)
 Heparin may be used cautiously by an expert hematologist.
(4) Correction of fluid & electrolyte balance.
(5) Adrenal hemorrhage in meningococcal meningitis may require
replacement therapy with adrenocortical steroids.
(6) Subdural effusions (visible on transillumination or CT scan) may occur in
infants & require repeated aspiration.
(7) Proper Nursing Care is mandatory to prevent aspiration pneumonia,
DVT, pulmonary embolism, UT infections & bed-sores.
N.B.: Prophylactic Antibiotic Therapy of Patient Contacts:
/ Rimactane 300 mg Cap.
Or: Rifadin 300 mg Cap.
(2 caps. every 12 hrs for 2 days in adults, & 10 mg/kg every 12 hrs for 2
days in children)
Follow up of Patients with Bacterial Meningitis:
(1) Postmeningitic seizures
(2) Postmeningitic eighth nerve damage with hearing impairment.
(3) Postmeningitic intellectual impairment.
TUBERCULOUS MENINGITIS







Fever, lethargy, headache, general malaise, behavioral & coqnitive
deficits, focal neurological signs, seizures, neck rigidity (in 75% of
patients) + Pulmonary T.B. (in 80% of cases).
Focal signs are due to exudate in the basal cisterns, ischemic infarcts
secondary to arteritis, intracranial tuberculomas. or hydrocephalus.
Chest X-ray may reveal pulmonary T.B.
CSF is turbid or cloudy, mononuclear cells (50 to 100 per mm 3), proteins
50 mg% > 200 mg% in 75% of pts.) glucose < half the blood glucose, &
low chloride content.
A positive acid-fast smear confirms the diagnosis.
CSF culture for mycobacteria takes 6-8 weeks.
Tuberculin test, ESR, serum electrolytes, liver enzymes, & CT scan of the
brain are helpful.
108
Treatment:
A triple drug therapy is recommended: (INH + Rifampin + Pyrazinamide)
/ Isocid Forte 200 mg Tab.
Or: Isocid 50 mg Tab.
(20mg/kg wt daily to a max. of 600 mg/day divided in 3 doses, for 8-12
wks Then reduce the dose to 10 mg/kg daily)
/ Rimactane 150 mg or 300 mg Cap
Or: Rifadin 150 mg or 300 mg Caps.
(15-20 mg/kg wt daily as a single dose 30 min before breakfast. Max.
600 mg/day)
/ Tebrazid 500 mg Tab.
(20-35 mg/kg wt, divided in 3 or 4 doses. Max. 3 gm/day)
/ Bedoxine Tab.
Or: Pyramine Tab.
(1 tab. daily)
N.B.:
 If CSF culture is sensitive to INH. discontinue Tebrazid. & continue on
INH & Rimactane for 18-20 months. If culture is negative or INH
resistance is clear, continue on triple therapy with the minimum effective
dosage for 2 years.
 Streptomycin (IM) may be given for 4 wks, (not exceeding 1 gm/day, in
conjunction with other antituberculous drugs) ‫أسابيك‬4‫ ًم بالعضع ياميا لمَة‬1
 Ethambutol (Etibi 250 mg or 500 mg Tab.) may be used in conjunction
with INH as a substitute for Tebrazid or Rimactane. (25 mg/kg/day as a
single dose)
Complications of T.B. Meningitis:
1. Hydrocephalus, increased ICP, & Coma.
Treated by: dehydration therapy & shunting.
/ Mannitol 20% Solution.
(1 gm / kg i.v. over 15-20 mn).
/ Decadron Vial. 8mg (0.5-1 mg / kg/d i.v. in 4 divided doses, for 10 days)
2. Epidural tuberculomas usually respond to medical therapy.
3. Pott's disease of the spine usually responds to medical therapy &
immobilization for 4 to 6 weeks.
4. Spinal radiculomyelitis: Medical therapy + Surgery in acute spinal cord
affection. Avoid surgery in patients with multiple cranial nerve palsies.
5. Intracranial tuberculoma is treated by triple-drug therapy. Surgery is
indicated if it leads to obstructive hydrocephalus.
109
VIRAL INFECTIONS OF THE CNS
HERPES SIMPLEX VIRUS ENCEPHALITIS
In Adults:
• Confusional state, fever, headache, seizures, focal deficits e.g. aphasia & /
or hemiparesis.
• Lesions are localized in the inferomedial portions of the temporal lobes, &
adjacent inferior portions of the frontal lobes on one or both sides +
cerebral edema.
In Infants:
• Herpetic vesicles on the skin few days after birth, poor feeding, jaundice
seizures & widespread virus-induced lesions in the brain.
Investigations:
1. EEG: usually shows temporal lobe epileptogenic abnormalities (periodic
spiking discharges)
2. CSF: pleocytosis, elevated protein, raised pressure ± RBCs & / or
xanthochromia.
3. CT scan of the brain: shows a low-density, irregular lesion in the medial
portion of the temporal lobe, with an irregular edge of mottled contrast
enhancement, & swelling of the temporal lobe. Such changes may take 5 or
more days to appear.
4. Radionuclide brain-scanning is positive early in the disease
5. MRI of the brain shows the characteristic lesion.
6. Brain Biopsy: histology, immunofluorescence microscopy, virus culture, &
electron microscopy confirm the diagnosis.
Treatment:
A. General Supportive Procedures: Nursing care of comatose patients e.g.
care of the skin, bladder, rectum, nutrition, fluid balance, respiration,...etc.
B. Drug Therapy:
• Antiviral Therapy:
/ Zovirax IV Infusion, 250 mg. (5 mg/kg i.v. infusion every 8 hrs)
Or: Vira-A Vials. 1000 mg. (15mg/kg i.v. infusion daily for 10 days)
• Dexamethazone for Brain Edema:
/ Decadron Vial, 8 mg/2ml.
Or: Fortecortin Amp.
(8 mg i.v. 6 hourly)
HERPES ZOSTER
• It is caused by the chickenpox virus (Varicella-zoster virus, VZV).
110
• VZV becomes latent in dorsal root ganglia, following a childhood
chickenpox. & is reactivated years later to produce H. Zoster.
• Clinically: radicular pain, vesicular skin eruptions on an erythematous
base, segmental sensory loss. focal paralysis, & rarely encephalitis.
• Postherpetic neuralgia: paroxysms of shooting, burning, stinging &
intolerable pain + hyperalgesia + depression.
Treatment:
1. Management of Acute H. Zoster:
• For Eradication of virus infection:
/ Zovirax 250 mg Vial. (5mg/kg by i.v. infusion every 8 hrs)
Or: Vira-A Injection. 1000 mg/5ml Vial.
(15 mg/kg by i.v. infusion daily for 5days at least)
/ Zovirax 5% Cream. (Apply locally 5 times daily for 5 days)
• For Prevention of Postherpetic Neuralgia:
/ Hostacortin 5 mg Tab. (60-80 mg, 12-16 tabs, daily for 2-3 wks)
• For Acute Pain:
/ Aspirin Tab. (2 tabs every 4-6 hrs)
/ Codeine Tab. (30-60 mg bid or tid)
2. Treatment of Postherpetic Neuralgia:
/ Tegretol 200 mg Tab.
Or: Tegral 200 mg Tab.
(Half tab. bid, gradually increased to 5-6 tabs. daily)
&/Or: Epanutin 100 mg Cap.
(3 caps. daily, gradually increased to 5-6 caps/day)
If unresponsive:
/ Tryptizol 25 mg Tab.
(1 tab. at night, gradually increased to 3-4 tabs. /day)
/ Neurontin 400 mg Cap. (1cap daily, gradually↑ to 1-2 caps tid for 5 - 10
days)
3. Treatment of Immunocompromised Patients Exposed to HZV:
/ Zovirax 250 mg Vial.(5 mg/kg by i.v. infusion every 8hrs. for 5-10 days)
/ Zovirax 5% Cream. (Apply locally 6 times daily for one week)
N.B.: Avoid steroids in immunocompromised patients.
111
MOVEMENT DISORDERS
PARKINSON'S DISEASE
Neurochemical Basis:
 Dopamine deficiency within the striatum → akinesia, rigidity. &
postural instability.
 Cholinergic abnormalities → tremor
 Norepinephrine abnormalities → orthostatic hypotension & depression
 Serotonin abnormalities → sleep disturbance
 Cholinergic cortical projections & cortical neurons abnormalities →
dementia.
Parkinsonism: is characterized by:
 Hyperkinesia: Static tremors.
 Hypokinesia or bradykinesia: Mask facies; slow monotonus speech;
slow, short-stepped, shuffling gait;... etc.
 Hypertonia (Rigidity): Plastic lead-pipe or cog-wheel rigidity.
 Autonomic disturbances e.g. postural hypotension, excessive salivation
& sebaceous secretion, impotence,... etc.
 Depression, & dementia.
Treatment:
Medications for Parkinson’s disease
Dopamine precursors
 Levodopa in combination with a dopa decarboxylase inhibitor
(Sinemet, Sinemet CR).
Dopamine receptor agonists
 Bromocriptine (Parlodel)
 Pergolide (Permax)
 Pramipexole (Mirapex).
 Ropinirole (Requip)
 Cabergoline (Dostinex, Cabaser)
 Lisuride (Dopergin)
Monoamine oxidase (MAO) B inhibitors
 Selegiline (Jumex, deprenyl, Eldepryl).
Amantadine: Amantadine HCl (Symmetrel, Amantine, Adamine)
Catechol-o-methyltransferase inhibitors
 Entacapone (Comtan)
Anticholinergics
 Trihexyphenidyl (Artane)
112
 Benztropine (Cogentin)
 Biperiden (Akineton)
 Orphenadrine (Disipal)
 Procyclidine (Kemadrin)
A. Treatment of Early Parkinsonism:
(1) Encourage regular activity & exercise (e.g. swimming, a stationary
bicycle, or a rowing machine)
(2) Emotional support
(3) Antiparkinsonian Drug Therapy:
/ Cogentin 2 mg Tab.
Or: Akineton 2 mg tab.
Or: Artane 2 mg Tab.
Or: Parkinol 2 mg Tab.
(1 mg daily for 1 wk., then gradually increase the daily dose by 1 mg
every wk. until symptoms are controlled. Max. 6 mg/day)
/ Adamine 100 mg Cap.
Or: Amantine 100 mg Cap.
(100 mg bid)
/ Jumex 5 mg Tab. (1tab. bid)
/ Sinemet CR 220 mg Tab. (Half tab. tid. Gradually increase the daily dose
by half tab. every wk until symptoms are controlled. Max. 4tabs/day).
B. Treatment of Late Parkinsonism:
/ Sinemet 275 mg Tab. (1/2-1 tab. 4-6 times daily)
N.B.: Lack of efficacy, or rapid fluctuation (the on-off response), add:
/ Parlodel 2.5 mg Tab (Half / tab. with meals bid. Gradually increase the
daily dose by 1.25 mg every wk. until symptoms are controlled. Max. 6
tabs/day)
/ Adamine 100 mg Cap.
Or: Amantine 100 mg Cap.
(1 cap. bid for 6 months, then discontinue for I month, then repeat the
course, discontinue for 1 month, & so on)
N.B.:
 If dyskinesias (choreoathetotic movements of the face, tongue. & limbs)
occur, reduce the daily dose of sinemet by 1 tablet.
 Delirium, hallucinations, confusional state ± agitation, & hypotension
may occur during the course of therapy & necessitate  the dose of
antiparkinsonian drugs, or a drug holiday (discontinuing sinemet for 5-7
days, then re-instituting it at a dose lower than the previous daily dosage)
C. Treatment of Problems Associated with Parkinsonism:
• Sleep disturbances, can be controlled by:
/ Tryptizol 25 mg Tab.
Or: Avil-Retard Tab.
(1 tab. at bedtime daily)
113
• Unpredictable Falls:
 The patient is advised to look forward & to avoid looking at his feet
during walking
 He can use crutches if necessary.
 He may need training focusing on: how to fall down safely & the way
of walking step by step, under the supervision of a physiotherapist.
• Dementia:
 It affects 25% of parkinsonian patients within the first 5 years, & 50%
after 10 years of the disease.
 Drug toxicity should be excluded as a cause of impaired memory
 Exclude subdural hematomas
 Reduce the dose of antiparkinsonian drugs, especially anticholinergics.
• Agitation:
 Reduce the dose of antiparkinsonian drugs
/ Melleril 30 mg Tab.
(30 – 60 mg at bedtime daily)
• Akathisia: (motor restlessness. & restless legs)
Usually responds to increasing the dose of Sinemet, or adding an
anticholinergic drug (e.g. Cogentin or Parkinol)
• Orthostatic Hypotension:
/ Astonin H Tab. (1 tab. bid until BP is stabilized, then 1 tab daily)
• Nausea or Vomiting:
 The patient is advised to take medicines during or after meals.
/ Prepulsid Tab
Or: Motilium Tab
(1 tab tid, a. c.)
• Depression:
/ Tryptizol 10 mg, 25 mg Tab. (1 tab. tid)
Or: Trittico 50 mg or 100 mg Tab.
(50-100 mg at bedtime)
± Electroconvulsive therapy (E.C.T.)
N.B.: Avoid MAO inhibitors in patients receiving Sinemet
• CHF, Angina Pectoris, Cardiac Arrhythmias, Hepatic Failure, Renal
Failure:
 Adjustment of the dose of antiparkinsonian drugs is needed.
• Prominent Parkinsonian Tremor ± Essential Tremor:
/ Inderal 10 mg or 40 mg Tab. (10-40 mg tid, before meals)
/ Cogentin 2 mg Tab.
Or: Akineton 2 mg Tab.
(1 mg every night, gradually increased to 1 tab. tid)
114
If unresponsive:
 Thalamotomy using a radio-frequency device may be helpful.
Surgical Treatment of PD
Ablative procedures:
 Thalamotomy
 Pallidotomy
 Subthalamotomy
Stimulation procedures:
 Thalamus (VIM nucleus)
 Globus pallidus internus (GPi)
 Subthalamic nucleus (STN)
Relative merits of different surgical procedures for PD:
Procedure
Tremor
Thalamotomy
Pallidotomy
DBS-thalamus
DBS-Gpi
DBS-STN
Fetal nigral
transplantation
+++
++
+++
++
+++
++
Rigidity/
Bradykinesia
+/++
+/+++
+++
++
Dyskinesia
+/+++
+/+++
+++
++/-
Adverse
events*
3
3
2
2
2
1
+ = mild benefit; ++ = moderate benefit; +++ = marked benefit
* For bilateral procedures, 1 = minimal risk; 2 = more pronounced risk; 3 = great risk
HUNTINGTON'S DISEASE (HD)


It is inherited through an autosomal dominant gene.
It presents with progressive intellectual deterioration (dementia) &
diffuse chorea.
 Two types are known: hyperkinetic-hypotonic & hypokinetic rigid.
Treatment:
A. Drug Treatment of Movement Disorders in Huntington’s Disease:
Chorea
 Responds to antidopaminergic agents, particularly conventional
antipsychotics or typical neuroleptics
 They may cause: extrapyramidal side effects, or tardive dyskinesia
Treatment:
 Typical Neuroleptics, OR: Atypical Neuroleptics (better tolerated)
115

Dopamine depletion or receptor blockade is helpful:
/ Haloperidol (Haldol, Safinace, Serenase),
(1.5 – 5 mg tid – qid)
Or: Chlorpromazine (Largactil, Neurazine),
(25 – 50 mg tid)
Or: Reserpine (Serpasil),
(0.25 - 0.5 mg qid) (in Sydenham’s chorea)
/ Propranolol (Inderal), in high dosage, (useful for action tremor)
(10 – 40 mg, a. c., tid)
/ Dantrolene sodium (Dantrium), for chorea + hemiatrophy
Dystonia
/ Trihexyphenidyl (Artane), (1-2 mg bid, ↑ up to 12-15 mg /d on tid
schedule)
Or: Baclofen (Lioresal), (5 mg bid ↑ up to 60-80 mg /d on tid or qid
schedule)
Or: Clonazepam (Rivotril), (0.25 mg bid ↑ up to 6 mg /d)
Or: Botulinum Toxin (Botox A)
 Blepharospasm: (30-50 U )
 Torticollis: (200 U or more)
Myoclonus (Brief shock-like jerks)
/ Valproate (Depakine), (250 mg tid, ↑ gradually to achieve a serum level
of 50-100 ug /ml)
/ Clonazepam (Rivotril), (0.25 mg bid, ↑ up to 6 mg /d)
Tics (Repetitive stereotyped motor or vocal behaviors)
/ Haloperidol (Safinace), (1.5 mg /d, ↑ up to 10-15 mg /d)
Or: Pimozide (Orap Forte), (1-2 mg /d, ↑ up to 10 mg /d)
Rigidity & Plasticity
/ Tizanidine (Sirdalud), (2 mg at bedtime, ↑ every wk to maximum of 12-24
mg /d)
Or: Diazepam (Valium), (2 mg tid, ↑ up to 10 mg tid-qid)
Or: Baclofen (Lioresal),10 mg & 25 mg Tab.
(5 mg bid, ↑ up to 60-80 mg /d. on tid or qid schedule
Hypokinesia, Parkinsonism
/ Levodopa / Carbidopa (Sinemet), 275 mg Tab.
(1/2 tab tid)
Seizures
 AEDs
116
B. Drug Treatment of Psychiatric Symptoms in Huntington’s Disease:
Mood Disorders
/ Venlafaxine (Effexor), (37.5 mg / morning with food ↑ to 75 mg / morning
up to 225 mg SR)
Or: Nefazodone (Serzone), (300 mg /d in divided doses)
Or: Mirtazapine (Remeron), (15-45 mg at bedtime)
Or: Bupropion (Wellbutrin), (75 mg orally / morning, ↑ to 100 mg /
morning , then ↑ to 100 mg bid-tid)
/ Methylphenidate (Ritalin), (5 mg every morning, ↑ to 5 mg morning &
noon, then ↑ by 5 mg every 3-4 days until response is achieved)
Anxiety Disorders
/ Buspirone (Buspar), (5 mg every morning, ↑up to 10-60 mg/d)
Or: Hydroxyzine (Atarax) (25 mg o.d.-qid)
Or: Alprazolam (Xanax), (0.5 mg tid)
Or: Bromazepate (Lexotanil, Calmepam), (1.5 mg tid)
Or: Clorazepate (Tranxene), (5 mg tid)
Aggression, Irritability, & Dyscontrol
/ Propranolol (Inderal), (10-40 mg tid-qid).
/ Lithium (Priadel), (300-1200 mg /d.)
Apathy
/ Methylphenidate (Ritalin), (5 mg every morning, ↑ to 5 mg morning &
noon, then ↑ by 5 mg every 3-4 days until response is achieved)
Psychosis
/ Risperidone (Risperdal) (1-6 mg daily)
Or: Haloperidol (Safinace) (5-15 mg daily)
Or: Olanzapine (Zyprexa) (5-20 mg daily)
Insomnia
/ Mirtazapine (Remeron), (15-45 mg at bedtime)
Or: Trazodone (Trittico),
(100-200 mg at bedtime)
Sexual Disorders
 Hypoactive sexual desire
 Paraphilic disorders


Inhibited orgasm
Inappropriately heightened
activity
They may respond to:
 Medroxyprogesterone acetate
117
sexual
 Leuprolide (Lupron) (Gonadotropin-RH agonist)
 SSRIs
ECT in HD:
 Effective in depression & abnormal movements & generally safe
Assistive Devices in HD:
 Wheelchairs + Specialized walkers
 Adaptive seating & positioning devices
 Bed enclosures & padding
 Tub seats
 Shower rails
 Ankle & wrist weights
 Weighted utensils
 Lidded cups
Physical / Speech Therapy & Exercises
 Physical & occupational therapies to keep safety & independence
 Using assistive devices for performance of A.D.L.
 Speech therapy
Genetic Counseling in HD
 DNA testing
 Diagnostic
 Predictive testing of asymptomatic individuals
Psychotherapy in HD
 Psychotherapy or supportive or insight-oriented therapy is helpful as an
adjunct to drug therapy
Emerging Therapies for HD
Pharmacologic Approaches:
/ Riluzole (Rilutek), 50 mg tab. (50 mg bid)
 N-methyl-D-aspartate (NMDA) receptor antagonist
 Reduce chorea in HD
/ Remacemide (NMDA glutamate receptor antagonist)
/ Coenzyme Q10 cap, (1 – 3 cap/d)
 ↑ Mitochondrial energy production
 Acts as an antioxidant
Surgical Approaches:
 Striatal transplantation
118
RHEUMATIC CHOREA (SYDENHAM'S CHOREA)
Involuntary, dysrhythmic, purposeless, nonstereotyped movements of' the
face, trunk & extremities, occurring at rest + Hypotonia → boat-shaped
hand & pendular knee jerk + Dysarthria ± Associated rheumatic fever or
rheumatic carditis + Raised ESR, CRP & ASOT.
Treatment:
/ Safinace 1.5 mg Tab. (Half-1 tab. daily, increased gradually to 1 tab. tid)
Or: Serpasil Tablets. 0.25 mg.
Or: Largactil 25 mg Tablets.
Or: Promacid Tablets. 25 mg.
Or: Neurazine 25 mg Tablets.
(One tablet t.d.s. or q.d.s.)
/ Aspocid Tablets.
(If no carditis)
(2-4 tablets after meals, t.d.s.)
Or: Hostacortin Tablets.
Or: Deltacortril Tablets.
Or: Predilone Tablets.
(In carditis, rheumatic fever or failure of other lines of treatment)
(One tablet q.d.s. for 5 days, then t.d.s. for 5 days, then b.i.d. for 5 days,
then once daily for 5 days).
/ Pentrexyl 250 mg Caps.
Or: Ampicillin 250 mg Caps.
(One caps. 6 hourly for one week).
/ Penadur. 1,200,000 U.
Or: Penicid L.A. 1,200,000 U.
Or: Durapen 1,200,000 U (i.m. every month)
/ Totavine Tablets
Or: Viterra Caps
Or: Multisis-V Tablets.
Or: Vi-Total Caps.
(One tablet or one caps. b.i.d.)
+ Treatment of co-existing rheumatic heart disease.
N.B.: Absolute bed rest is necessary until the pulse& E.S.R. become normal.
119
TREMOR
Involuntary, regular, & repetitive shaking of a body part around a fixed point
Characteristics of Major Tremor Syndromes:
Parkinsonian
Essential
Cerebellar
Rubral
Asterixis
Physiological
++
pill-rolling
0
+
+
+
0
Sustained
position
+
++
-
++
Sway
++
Irregular
+
Intention
+
+
++
++
+
0
Tremor
during
sleep
0
0
+
+
+
0
Agonist
&
Antagonist
contractions
Agonist &
antagonist
contractions,
asynchronous
&overlapping
Mixed
pattern
Loss of
activity
Alternating
agonist &
antagonist
contractions
8-12 cps
Clinical
Rest
EMG
Alternating
agonist &
antagonist
contractions
3-7 cps
essential
tremor also
seen
Classification of Tremors
Action Tremors: (7-12 cps, of low amplitude, may be asymmetric)
They include:
 Physiologic tremor: induced by movements, requiring extremes of
precision or power, ↑ by fatigue, anxiety, weakness, hypercapnia, drug
withdrawal, hypoglycemia, uremia, cholemia, thyrotoxicosis, heavy
metal intoxication, & drugs e.g. catecholamines, amphetamine,
theophylline, caffeine, lithium, tricyclic antidepressants, antipsychotics,
sodium valproate
 Familial tremor
 Senile tremor in the elderly
 Essential tremor if no demonstrable cause
Essential Familial Tremor
 It is a monosymptomatic action tremor affecting the upper limbs. neck,
face, & voice, with a positive family history.
120

It is temporarily ameliorated by ingestion of small amounts of ethylalcohol, with a rebound enhancement as blood levels of alcohol fall.
 It may be associated with parkinsonism in some patients.
Treatment of Essential Familial Tremor:
A. Drug Therapy:
/ Mysoline 250 mg Tab.
OR: Mysoline Susp., 250 mg/5 ml
(125 mg at bedtime daily, gradually increased by 125 mg every wk. until
tremor is controlled. Max. 250 mg tid)
If ineffective or intolerable substitute by:
/ Inderal Tablets, 10 mg or 40 mg (10-20 mg tid-qid, gradually increased
until tremor is controlled. Max. 40-80 mg tid-qid)
B. Surgery:
Stereotaxic ventrolateral thalamotomy is curative in more than 80% of
cases.
Enhanced Physiological Tremor
 An independent action tremor seen with anxiety, nervousness, &
adrenergic over-activity.
 Tremor is enhanced by adrenergic drugs, tricyclic antidepressants,
thyrotoxicosis, chronic anxiety, & hypoglycemia.
 Tremor is controlled by propranolol (Inderal) ± an anxiolytic
/ Inderal Tablets. 10 mg or 40 mg.
(40-80 mg 1 hr before exposure to stress + 10-40 mg tid)
± / Valinil 2 mg Tab.
Or: Xanax 0.5 mg Tab.
(1-3 tabs. daily)
 Thyroxine therapy, anti-asthmatic adrenergic agents, caffeine & other
xanthines, amphetamines, lithium, tricyclic antidepressants, &
hypoglycemia, exaggerate physiological tremor & need to be controlled
or adjusted.
Orthostatic Tremor:
 Unsteadiness while standing but steadiness while walking.
 Treated by:
/ Clonazepam (Rivotril),( 0.5-1 mg/day)
Asterixis:
 The rate of limb flexion & extension is irregular & slow
 It arises from temporary loss of tone in the outstretched limb
 Occurs in metabolic disorders (renal, pulmonary, hepatic), in Wilson’s
disease, & with some drugs e.g. metoclopramide (Plasil) &
anticonvulsants
121

Treatment is that of the cause
CEREBELLAR ATAXIA
Cerebellar Neurotransmitters:
Afferent pathways to cerebellar cortex & deep cerebellar nuclei
 Mossy fibers from the pons - (glutamate, acetylcholine, various neuropeptides)
 Climbing fibers from the inferior olives - (aspartate, corticotropin
releasing factor)
 Noradrenergic fibers from the locus ceruleus
 Serotinergic fibers from the raphe nuclei
 Histaminergic fibers from the upper brain stem & hypothalamus
Cerebellar cortical neurons
 Purkinje’s cells, basket cells, stellate cells, Lugaro cells, Golgi cells(GABA) (both GABA-A/benzodiazepine & GABA-B/baclofen receptors
present)
 Granule cells-(glutamate) (interacting with non-NMDA type receptors)
 Deep cerebellar nuclei & associated efferent pathways
- To brain stem, red nucleus, & thalamus-(glutamate)
- To inferior olive-(GABA)
Treatment of Cerebellar Ataxia:
Diet & Lifestyle:
 Vegetarian diet may predispose to Vit. B12 deficiency which could
complicate ataxia
 Deficiencies of thiamine (B1), Vit.B12, & Vit. E can cause ataxia
Drug Treatment:
/ Amantadine (Amantine, Adamine) Cap.
 A tricyclic amine that ↑ release of dopamine & other monoamines
(serotonin & noradrenaline)
 Start with 100 mg /d
 ↑ biweekly to b.i.d. or tid
 Children: ½ adult dose (start with 50 mg /5 ml syrup)
/ L-5 HTP
 A precursor of serotonin, may be effective in mild & moderate
ataxia
/ Buspirone (Buspar) 5 mg or 10 mg Tab.
 A serotonin 5-HT1A receptor agonist
 Start with 5 mg o.d. or bid
122
↑ biweekly by 5 mg to maximum of 20 mg tid
Usually 10-30 mg /d are enough
/ Acetazolamide (Diamox, Cidamex) 250 mg Tab.
 A brain carbonic anhydrase inhibitor CNS acidosis
 Improves episodic ataxia & vertigo
 Start with 250-500 mg /d.
 ↑ up to 1000-4000 mg /d. as tolerated
/ Benzodiazepines e.g. Clonazepam (Rivotril) 0.5 mg or 2 mg Tab.
 A GABA-A receptor modulator Can reduce cerebellar tremor
 0.5-6 mg /d.
 May lead to long-term worsening of balance & incoordination
/ Baclofen (Lioresal) 10 mg & 25 mg Tab.
 A GABA-B agonist
 Improves spasticity, but may worsen ataxia
/ Physostigmine (Prostigmine, Nestigmine, adcostigmin, Amostigmin)
15mg Tab.
 A centrally acting acetylcholinesterase inhibitor
 Oral & s.c. use may be useful
/ Isoniazid (INH) Tab.
 A GABA transaminase inhibitor
 May be useful in kinetic tremor
 Can cause an acute cerebellar syndrome
/ Propranolol (Inderal)10-40 mg Tab.
 A beta-blocker
 Effective for postural tremor
/ Primidone (Mysoline) 250 mg Tab or 250mg/ 5ml syrup
 A barbiturate AED
 Effective for postural tremor, but may worsen ataxia
/ Carbamazepine (Tegretol), 200 mg Tab.
 400-600 mg /d.
 Improves cerebellar tremor
/ Gabapentin (Neurontin) (400 mg tid.)
/ Baclofen (Lioresal) can improve nystagmus, but worsen ataxia
(10 – 25 mg tid)
/ Clonazepam (Rivotril) 0.5 mg or 2 mg tab
/ Fluoxetine (Prozac) 20 mg cap. improves pseudobulbar affect & motor
dysfunction (e.g. dysarthria , dysphagia for liquids)
(20 mg in the morning)


123
/ Botulinum Toxin Type A (Botox A) controls head nodding & tremor
(15-25 U per muscle)
Surgical Treatment:
 Thalamotomy: controls tremor unresponsive to drugs
 Thalamic stimulation: is better tolerated
Physical / Speech Therapy, Exercise:
 Rehabilitation: safe ambulation & independence in ADL
 Assisted exercise, gait training, speech & swallowing training, use of
assistive devices, … etc.)
 Exercise & biofeedback training
 Central vestibular compensation exercises
Other Treatments:
 Alternative therapies (Complementary Medicine) e.g. acupuncture,
chiropractic may be helpful in pain relief
 Megavitamin therapy may be harmful:
 Excessive Vit. B6 intake may → peripheral neuropathy
 Excess iron accumulates in neuronal mitochondria in deep
cerebellar nuclei in pts with Friedreich's ataxia gene → free radical
damage → neuronal cell death
 Megadose Vit .C therapy may → absorption of dietary iron
Emerging Therapies:
 Antioxidants e.g. Vit.E, Coenzyme Q10, Idebenone , Riluzole (Rilutek)
 N-acetyl-cysteine
 A free radical scavenger, improves speech, coordination & gait
 500-1500 mg tid
124
PERIPHERAL NERVE DISEASES
POLYNEUROPATHIES
CAUSES OF POLYNEUROPATHY
I. Heredofamilial Polyaienropathies :
1. Peroneal Muscular atrophy.
2. Hypertrophic interstitial polyneuropathy.
3. Hereditary sensory radicular neuropathy.
4. Refsum's disease.
II. Infective :
1. Acute infective polyneuropathy (Guillain-Barre syndrome).
2. Diphtheritic polyneuropathy.
3. Leprotic polyneuropathy.
4. Systemic fevers e.g. malaria, measles, meningitis, mumps, scarlet
fever, septicaemia, smallpox, syphilis, typhoid, typhus, tetanus,
paratyphoid, dysentery, gonorrhea, influenza.
III. Toxic :
1. Metals: Lead, antimony, arsenic, mercury, copper,...etc.
2. Organic substances & Drugs: CO, C CU, chloroquine, chloral, triorthocresyl-phosphate, sulphonamides, steroids, streptomycin, I.N.H.,
cycloserine, Furadantin & immune sera.
IV. Nutritional Deficiency :
1. Beriberi.
2. Pellagra.
3. B12 deficiency.
4. Folic acid deficiency.
5. Alcoholism.
6. Pregnancy.
7. Steatorrhoeas & G.I.T. operations.
V. Metabolic & Endocrinal :
1. Diabetes.
2. Porphyria.
3. Uraemia.
4. Myxoedema.
5. Acromegaly.
6. Amyloid disease.
VI. Vascular :
1. Arteriosclerosis.
2. Polyarteritis nodosa.
3. Thromboangiitis obliterans.
125
VII. CoIlagen Disorders:
1. Disseminated lupus erythematosus.
2. Rheumatoid arthritis.
3. Scleroderma.
4. Sarcoidosis.
VIII. Polynenritis of obscure origin :
1. Carcinomatous neuropathy.
2. Chronic progressive polyneuritis.
3. Recurrent polyneuritis.
4. Myelomatosis.
CRITERIA OF DIAGNOSIS OF SPECIFIC POLYNEUROPATHIES:
Criteria of Diagnosis of Peroneal Muscular Atrophy:
1. Bilateral Asymmetrical peripheral muscular muscular weakness & wasting
with "inverted champagne bottle appearance" in L.Ls. & less marked
affection of U.Ls.
2. Marked discripancy between the degree of muscular wasting & weakness
i.e. marked wasting with only very mild weakness.
3. Hyporeflexia or areflexia in the wasted muscles.
4. Glove & stocking hypoesthesia + Loss of Vibration sen seat the ankles,
5. High-stepped Gait due to foot drop.
6. Pes cavus & talipes equinovarus.
7. Plantar reflexes are lost.
Criteria of Diagnosis of Diphtheritic Neuropathy:
1. Bilateral Palatal paralysis with nasal tonation, nasal regurgition & lost
palatal reflex.
2. Bilateral paralysis ofaccomodation.
3. External ophthalmoplegia e.g. lateral rectus paralysis.
4. Generalised polyneuritis.
5. ± Cardiac paralysis.
Criteria of Diagnosis of Leprotic Neuropathy:
1. Maculo-anesthetic patches.
2. Polyneuritis with distal atrophic paralysis of the limbs, glove & stocking
anesthesia, L.M.N. facial palsy, thickening of the peripheral nerves e.g.
ulnar, lateral popliteal & greater auricular nerves + Trophic changes in the
limbs with ulceration & necrosis of the phalanges & loss of fingers.
3. ± Hyperreflexia due to lepromatous infiltration of the cord.
Criteria of Diagnosis of Lead Neuropathy:
1. Bilateral wrist & finger drop.
2. Occasional affection of the L.Ls.
126
3. No sensory changes.
4. Other symptoms: Blue line on the gums, constipation, colic, punctuate
basophilia & anemia, cardiac hypertrophy & hypertension & chronic
nephritis.
Criteria of Diagnosis of Diabetic Neuropathy:
1. Mononeuritis: e.g. lateral popliteal, femoral, sciatic, ulnar, median or 3rd,
6th & 7th nerve palsies.
2. Polyneuritis affecting the L.Ls. more than U.Ls. Sensory more than motor.
3. ± Diabetic Amyotrophy (Femoral nerve neuropathy) ± Diabetic
pseudotabes. ± Optic atrophy.
4. Manifestations of Diabetes Mellitus.
Criteria of Diagnosis of Alcoholic Neuropathy:
1. Sensory Poly neuropathy.
2. High-stepped gait,
3. ± 7th & 10th nerve affection.
4. ± Nystagmus.
5. Tender calf muscles.
GUILLAIN-BARRE SYNDROME (GBS)
It is a predominantly motor polyneuropathy of an acute or insidious onset, &
unknown etiology. Two thirds of cases are preceded by a minor illness e.g.
URI, an infection, or trauma.
Diagnostic Criteria:
I. Features Required for Diagnosis:
A. Progressive motor weakness or paralysis of the legs or of the 4 limbs
& trunk ± bulbar & facial paralysis, & external ophthalmoplegia.
B. Areflexia allover the 4 limbs, or distal areflexia with proximal hyporeflexia.
II. Features Strongly Supportive of Diagnosis:
A. Clinical Features:
1. Progression of motor weakness over 4 weeks.
2. Relative symmetry of signs & symptoms on both sides
3. Mild sensory symptoms or signs.
4.Cranial nerve affection e.g. bilateral facial weakness (in 50% of cases)
± bulbar palsy ± external ophthalmoplegia.
5. Recovery starts 2-4 weeks after progression stops.
6. Autonomic dysfunction e.g. tachycardia, arrhythmias, postural
hypotension, hypertension & vasomotor symptoms.
7. Absence of fever at the onset of neuritic symptoms.
127
Variants:
 Febrile onset
 Severe sensory loss with pain
 Progression beyond 4 weeks.
 Cessation of progression without recovery or with major residual
deficit.
 Transient bladder paralysis during evolution of symptoms
 CNS involvement e.g. cerebellar ataxia, dysarthria, extensor plantar
responses. & ill-defined sensory levels.
B. CSF Features Strongly Supportive of Diagnosis:
1. Elevated CSF protein after the first week of symptoms
2. CSF cells: 10 or fewer mononuclear leucocytes / mm3.
Variants:
 Normal CSF protein
 CSF cells of 11 to 50 mononuclear leucocytes / mm3.
C. Electrodiagnostic Features Strongly Supportive of Diagnosis:
1. Evidence of nerve conduction slowing or block in 80% of cases
2. NCV is usually less than 60% of normal
3. Distal latencies may be prolonged to 3 times normal
4. Normal NCV in 20% of cases.
N.B.: Other causes of acute or subacute neuropathy should be excluded e.g.
acute intermittent porphyria, diphtheritic neuropathy, lead neuropathy &
other toxic neuropathies (e.g. from furantoin, dapsone, organo-phosphorus
compounds, or abuse of or exposure to volatile solvents & vapors). Also
poliomyelitis, botulism, & hysteria should be excluded.
Prognosis:
• Improvement occurs in 80-85% of patients
• Mortality rate is 3-5%
• Cause of death is cardiac arrhythmias or pulmonary embolism
Treatment:
 IV IG ( 0.4 g/kg body wt daily for 5 days)

Plasmapheresis: is helpful if performed early (within the first 2 weeks of
onset of symptoms)
 Steroids: are of doubtful value
 Care of Respiration:
(1) 30-40% of paralysed patients require respiratory assistance.
(2) Vital capacity should be monitored
(3) Intubation + Ventilator (IPPV)
128
(4) Tracheostomy If a respirator is needed for more than 7 to 10 days.
 Motor Weakness or Paralysis:
(1) Careful positioning of the paralysed limbs in the optimal position for their
function.
(2) Passive exercises
(3) Splints
(4) Testing muscle power every 2 or 3 days
(5) Active exercises as soon as possible.
 Autonomic Instability:
 Fluctuations of BP & cardiac arrhythmias are frequent. BP & ECG
monitoring is necessary in such cases.
 Valium 2-5 mg orally may be enough to control hypertension.
 Cardiac arrhythmias need appropriate treatment.
 Pain: is controlled by analgesics
/ Surgam 300 mg Supp.
Or: Voltaren Supp.
(1 supp. every 12 hrs)
Or: Aspirin Tab.
Or: Paramol Tab.
(2 tabs. every 4-6 hrs)
 Reassurance
N.B.: Mini-dose heparin may be given to prevent DVT & pulmonary
embolism.
/ Calciparine 5000 U Amp.
(1 amp. s.c. every 12-24 hrs)
TREATMENT OF POLYNEUROPATHIES:
I. Hereditary Polyneuropathies:
No specific treatment
1. Hereditary Sensory Neuropathy
Physiotherapy, occupational
2. Chronic Hypertrophic Interstitial
therapy & rehabilitation may help.
Polyneuropathy
3. Peroneal Muscular Atrophy
4. Refsum's disease (hereditary neuropathy + ataxia + retinitis pigmentosa
+ elevated serum phytanic acid level)
Treated by : A diet low in phytanic acid ± Plasma exchange
5. Acute Intermittent Porphyria (acute proximal motor neuropathy +
abdominal pain + seizures + psychosis + delirium + autonomic instability + elevated urinary porphobilinogen)
Treated by:
/ Glucose infusions (300-400 gm/day)
/ Neurazine 25 mg Tab.
Or: Sparine Inj. (For pain)
129
(1 tab tid or 1-2 cc i.m. prn)
/ Inderal 10 mg or 40 mg Tab. (For autonomic symptoms)
(10-40 mg tid)
N.B.: Hematin therapy needs further evaluation.
6. Adrenomyeloneuropathy (Both upper & lower motor neuron features + a
disorder of fatty acid metabolism).
Treated by: Ganglioside
7. Metabolic storage disorders e.g. Leukodystrophies
Treated by: Bone marrow transplants.
II. Acquired Polyneuropathies:
Leprotic Neuropathy
Thickening of ulnar, lateral popliteal, & / or greater auricular nerves,
atrophic peripheral neuropathy with glove & stocking anesthesia, trophic
changes, maculo-anesthetic patches on the trunk, bilateral LMN facial palsy
Treated by:
/ Dapsone 50 mg Tab. (25 mg twice weekly, gradually increased, over 2
months, to 100 mg twice weekly)
± / Hostacortin 5 mg Tab. .
(for leprosy reactions)
(12 tabs. daily)
Diabetic Neuropathy
Distal symmetric sensory motor neuropathy, Proximal motor neuropathy,
Autonomic neuropathy, Mixed type, Painful diabetic neuropathy:
Treated by:
 Adequate control of diabetes is mandatory
 Pain is controlled by:
/ Tegretol 200 mg Tab. (600-1200 mg/day)
± / Epanutin 100 mg Cap. (300-600 mg/day)
± / Tryptizol 25 mg Tab. (50-150 mg/day)
± / Moditen 1 mg Tab. (2-5 mg/day)
± Transcutaneous nerve stimulator
 Orthostatic hypotension: Waist-high elastic stockings & Salt loading
/ Astonin H 0.1 mg Tab. (1 tab. o.d.-bid)
/ Indocid 25 mg Cap. (75-150 mg/day)
 Hypertension is controlled by antihypertensive drugs
 Bladder dysfunction needs self-catheterization
 Impotence is treated by Sildenafil (Viagra) or penile prosthesis in
resistant cases.
Vitamin Deficiency Neuropathies:
1. Alcoholic Neuropathy:
130




Abstinence of alcohol
Good nutrition
Control pain: using the regimen given for diabetic pain
Vitamin B1 + Multivitamin supplementation:
/ Neurobion Amp. (1 amp i.m. daily)
Or: Parentrovit IV Double Amp. (1 amp. i.v. daily)
2. Thiamine (Vitamin B1) Deficiency:
 Vitamin B1 deficiency leads to peripheral neuropathy (beriberi). Korsakoff psychosis & Wernicke's encephalopathy.
 It results from inadequate intake, alcoholism, malabsorption or protracted
vomiting.
/ Vitacid B1 Amp. 100 mg
Or: Benerva 100 mg Amp.
(One amp. I.M. daily until the condition improves)
Then maintain on:
/ Vitacid B1 Tablets 300 mg
Or: Neurobion Tablets.
(One tablet daily)
3. Subacute Combined Degeneration of the Spinal Cord (B12 Deficiency)
 Vitamin B12 deficiency may lead to pernicious anemia + subacute
combined degeneration of the spinal cord (pyramidal & posterior column
dysfunction) + peripheral neuropathy ± optic atrophy ± dementia.
/ Betolvex 1000 mcg Amp.
Or: Depovit-B12 1000 mcg Amp.
Or: Rubivamine 1000 mcg Amp. Or: Vitacid B12 Forte 1000 mcg Amp.
(One amp. I.M. daily for 2 weeks, then one amp. I.M. every month for
the rest of the patient's life).
4. Pyridoxine (Vitamin B6) Deficiency: (e.g. Isoniazid Neuropathy)
 Vit. B6 deficiency can lead to peripheral neuropathy (the burning feet
syndrome), optic neuropathy & convulsions. It can result from administration of antagonists e.g. isoniazid & penicillamine, malabsorption e.g.
coeliac disease & tropical sprue, or dietary insufficiency.
/ Vitamine B6 50 mg Amp.
Or: Ecovit B6 100 mg Amp.
(One amp. I.M. daily until the condition improves)
Then maintain on:
/ Bedoxine Tablets
Or: Pyramine Tablets.
Or: Trivarol Tablets.
Or: Tri-B Tablets.
(1-2 tabs daily)
131
5. Nicotinic Acid Deficiency (Pellagra)
 Nicotinic acid deficiency leads to pellagra which presents with dermatitis
of the exposed parts of the body + diarrhea + dementia + depression or
mania + peripheral neuropathy + pyramidal signs & symptoms.
 It may result from inadequate dietary intake or malabsorption.
/ Nicobion 500 mg Amp. & Tablets. 50 mg & 500 mg
(One amp. I.M. every 24 hours until improvement occurs, then tablets
are used 100-500 mg daily)
Or: Nicotinic Acid Tablets. 100 mg.
(One tablet t.d.s. until the condition improves, then half-1 tablet daily
afterwards)
/ Supravit Caps.
Or: Vi-Total Caps.
(1-2 caps. daily)
6. Folic Acid Deficiency
 Folic acid deficiency may lead to megaloblastic anemia, organic brain
syndrome, dementia, myelopathy & peripheral neuropathy.
 It can be induced by anticonvulsants e.g. phenytoin (Epanutin).
/ Folic acid Tablets. 5 mg. (One tablet tid or qid.)
Toxic Neuropathies:
 Heavy metals e.g. mercury, arsenic, thalium, lead.
/ Artamin 250 mg Cap. (1 cap. qid)
 Drugs e.g. vincristin, Nitrofurantoin: Drug-induced neuropathies usually
resolve after discontinuing the offending drug.
 Solvents e.g. hexacarbon solvents: They usually continue to deteriorate
for several weeks after removal of the toxin.
Neuropathy Associated with Cancer:
 Large-fibre sensory neuropathy (ataxia, areflexia, diminished vibration &
position sense, & loss of sensory action potentials). The neuropathy rarely
responds to treatment of cancer.
Vasculitis (e.g. systemic lupus erythematosus, polyarteritis nodosa, rheumatoid arthritis, & IV drug abuse).
 Rapidly progressive polyneuropathies or mononeuropathy multiplex.
/ Hostacortin 5 mg Tab.
(60 mg daily for 1 month, then every other day)
/ Endoxan 50 mg Tab. (2 mg/kg daily for 6 months at least, then gradually
reduce the daily dosage by 25 mg every month)
Other Systemic Diseases:
 Uremic Neuropathy: responds to: Hemodialysis. or Renal transplant
132

Hypothyroid Neuropathy: responds to: Thyroid replacement therapy:
Or: Tyraxin 0.1 mg Tab.
(1-3 tabs. daily)
Acromegaly may produce carpal tunnel syndrome, a mild senso-rimotor
polyneuropathy, or myopathy. They respond to treatment of the pituitary
lesion.
Amyloidosis causes both sensorimotor & autonomic neuropathy.
Treatment is symptomatic.
Tic paralysis: rapidly responds to removal of the tic.
/ EItroxin 0.1 mg Tab.



NEUROMUSCULAR JUNCTION DISEASES
MYASTHENIA GRAVIS (MG)









MG is a neuromuscular disorder characterized by weakness &
fatigability of skeletal muscles on repetition of muscular activity &
improvement by rest.
Ocular manifestations include: ptosis, diplopia, ophthalmoplegia.
Bulbar manifestations include: dysarthria, hoarseness of voice with
nasal tone, dysphagia & nasal regurgitation, & shortness of the breath.
Weakness of the proximal limb muscles most evident during hair
combing.
A descending march of symptoms is common.
Diurnal variation with worsening of symptoms in the afternoon is
characteristic.
Worsening of symptoms may occur with menstruation, infection, hyperor hypothyroidism, drugs e.g. aminoglycosides, penicillamine, &
antiarrhythmics.
Clinical Tests that help in diagnosis include:
 Forward arm abduction time
 Walker's test (Applying a sphygmomanometer cuff to the arm, raising the pressure to above systolic, exercising the fingers until
exhaustion, and then releasing the cuff → ptosis.
 Maintaining an upward gaze → ptosis
 Counting from 1 to 50 → dysarthria & nasal tone
 Repetition of the knee-jerk → fatigability.
Pharmacological Tests include:
(1) Tensilon test: 2 mg i.v. to test for idiosyncrasy, then 8 mg i.v. →
improvement of ptosis within 2 min.
133
(2) Prostigmine test: 2.5 mg i.m. + 1 amp. atropine → improvement of
ptosis within 10 to 30 min.
 Repetitive supramaximal nerve stimulation → decrement of more than
15% at 3 Hz stimulation.
 Anti-Acetylcholine Receptor (Anti-AChR) Antibody Test is positive in
more than 80% of patients.
 Muscle biopsy →  number of AChRs per neuromuscular junction.
 Other investigations include: thyroid function tests, pulmonary function
tests, tuberculin test, rheumatoid factor, chest x-ray, FBS, & CT scan of
mediastinum (to detect possible thymic enlargement)
N.B.:
 Anti-AChR antibodies are produced by the B lymphocytes.
 Over 75% of myasthenic patients have thymic hyperplasia (85%) or
thymoma (15%).
 About 5% of myasthenic patients have thyrotoxicosis.
Treatment of MG:
I- Cholinesterase Inhibitors:
 Start low, go slow
 Keep the patient slightly under-medicated
 Begin Pyridostigmine (Mestinon) at 60 mg tid, ↑ the dose progressively
to achieve the best response, or until the patient becomes cholinergic
(develop diarrhea)
II- Thymectomy:
 Thymectomy is recommended in all patients with generalized MG whose
symptoms began before age 50
 Thymectomy is recommended in children with MG if they have
functionally disabling weakness despite adequate treatment with
cholinesterase inhibitors
III- Immunosuppressive Therapy (Immunotherapy):
 Immunotherapy in MG is usually a lifetime commitment
Immunosuppressants in MG:
 Prednisone (Hostacortin) (Initially 60 mg/d; Maintenance 10 mg/d.)
 Azathioprine (Imuran) (150 mg/d., 50mg tid) [Monitor L.F.Ts.& CBC]
 Cyclosporine (Sandimmun) (150 mg bid) [Monitor R.F.Ts.]
 Prednisone +Azathioprine/Cyclosporine
 IV immunoglobulin (IVIG) → Rapid, temporary improvement of MG
 Plasma Exchange (PLEX) → Rapid, temporary improvement of MG
 50% of pts with ocular myasthenia & 20% of those with generalized MG
134
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



are seronegative (i.e. have no AChR-Ab)
The serum AChR-Ab level is not useful in monitoring the effect of
treatment
Ocular Myasthenia Gravis responds best to prednisone therapy.
Neonatal Myasthenia Gravis: occurs in 1 of 6 newborns to mothers with
MG. & is characterized by weakness of sucking & crying, dysphagia &
dyspnea ± generalized weakness. Treated by: Prostigmine, 0.05-0.1 mg
IV every 3 to 4 hrs until oral Mestinon (4-10 mg / 4 hrs) can be taken.
During pregnancy, Prostigmine is given IV (1 mg is equivalent to 60 mg
Mestinon). & Parenteral steroids (e.g. Hydrocortisone or Solu-Medrol
IV) are also used for 2 to 6 days postoperatively)
Curare-like drugs should be avoided in MG.
CONGENITAL MYASTHENIC SYNDROMES
Criteria of diagnosis:
Myasthenic symptoms & signs in a child:
 Neonatal myasthenia (if the mother has MG)
 Autoimmune acquired MG (is seronegative in 50%, i.e. have normal
AChR-Ab)
 EMG → myasthenic reaction
 AChR-Ab are elevated in 50% of cases
D.D. of Congenital Myasthenic Syndromes:
 Neonatal myasthenia:
 The mother has MG
 Improves spontaneously with supportive care
Treatment:
 Cholinesterase inhibitors.
 3,4- Diaminopyridine (DAP).
 Ephedrine.
NEONATAL MYASTHENIA




It occurs in 20% of babies born to myasthenic mothers
It is transitory & is usually gone in 24-36 hrs.
Symptoms usually begin within 3 days of birth
Signs include: mask-like face, poor sucking, difficulty swallowing,
regurgitation, & resp. distress
135
Treatment:
 Symptomatic, to prevent aspiration, provide nutrition, & maintain
respiration
/ Neostigmine (Prostigmine),
1-2 mg PO (Parenteral dose is 1/30 the oral dose) q4hrs
Or: Pyidostigmine (Mestinon), 4-10 mg PO, may be needed for a short
time
Treatment of MG in Pregnancy






MG may become worse, improve, or remain unchanged during
pregnancy
Therapeutic abortion is not indicated. Spontaneous abortion is common
in the first trimester.
Labor is shorter in myasthenics. IM anticholinesterases are used during
labor
Local or regional anesthetics are preferred to general anesthesia, &
caution is required with sedatives
C.S. is done only for obstetric indications
Contraception: Voluntary sterilization or contraception should be
suggested in severe MG.
MYASTHENIC CRISIS & CHOLINERGIC CRISIS
Myasthenic Crisis
Myasthenic patients may develop a dangerous degree of paralysis of
respiration or swallowing (dyspnea or dysphagia), & they are said to be in
"crisis".
Treatment:
 Ensure an adequate airway.
 Postural drainage & suction of pharyngeal secretion.
 Artificial respiration by mouth-to-mouth breathing.
 Intubation by a cuffed endotracheal tube & connection to a mechanical
ventilator for intermittent positive pressure breathing.
 Plasmapheresis if available. It removes antibodies & other circulating
factors from the patient's serum.
 Tensilon Amp. 10 mg (Edrophonium HCl)
136

(2 mg i.v. initially to test for hypersensitivity, if tolerated, inject the
remaining 8 mg. Improvement occurs in half-1 min. & continues for 5
min. To be repeated as needed)
Prostigmine inj. (Neostigmine)
(2.5 mg s.c. or i.m. It acts in 15-20 min)
+ Atropine Sulfate Amp. (0.6 mg s.c. or i.m.)
Cholinergic Crisis
Increasing paralysis due to overdosage with anticholinesterase drugs e.g.
Neostigmine (Prostigmine), Pyridostigmine (Mestinon), & Ambenonium
(Mytelase). Other parasympathomimetic signs may occur e.g. diarrhea, colic,
salivation, sweating, & bronchospasm, as well as fasciculation, and
constricted pupils.
Treatment:
 Discontinue all anticholinesterase medication
 Intermittent positive pressure ventilation (IPPV) through a cuffed
endotracheal tube.
 Atropine Sulfate Amp.
(2 mg i.v. every hr. until signs of atropine toxicity develop e.g.
confusion, hot flushed face, dry mouth, dilated fixed pupils, tachycardia,
urinary retention)
LAMBERT- EATON MYASTHENIC SYNDROME (LEMS)

It is an autoimmune disorder characterized by proximal muscle
weakness, hyporeflexia, & autonomic symptoms e.g. dry mouth.
 It may occur as a remote non-metastatic manifestation of oat cell
carcinoma of the lung, & may precede overt signs of the tumor by 4
years or more.
 It may also occur with other autoimmune diseases, or as an isolated
disorder.
 An IgG antibody binds to nerve terminals concerned with transmitter
release, thus reducing acetylcholine (ACh) release.
 EMG: Repetitive supramaximal stimulation of a motor nerve produces
increase in the amplitude of the evoked potentials (Eaton-Lambert
reaction)
Criteria of diagnosis of LEMS:
 Clinical evidence of malignancy
 Chest radiographs & CT scan reveal bronchogenic carcinoma
137
 Bronchoscopy may confirm the presence of bronchogenic carcinoma
 EMG → initial incremental response
D.D. of LEMS: MG
Treatment of LEMS:
 Effective cancer therapy
 Pyridostigmine (Mestinon), (30-60 mg q4 to 6hrs,) &
 Guanidine HCl, (5-10 mg /kg / day orally, divided throughout the
waking hrs). Side Effects: Bone marrow depression; renal, hepatic. &
pancreatic abnormalities (Monitor CBC, LFTs, RFTs.); AF &
hypotension; skin rash; neurological symptoms (paresthesias, ataxia,
confusion, & mood change)
OR:
 4-aminopyridine (Fampridine)
 It increases the number of quanta released by a nerve impulse
 Dosage: 1.25 mg / kg / day orally in divided doses at 3 or 4-hr
intervals during the day.
 Side Effects: Convulsions, confusion, agitation, insomnia,
paresthesias, & joint arthralgias.
OR:
 3,4-diaminopyridine
 It has the same mode of action as that of 4-aminopyridine, but is 6-7
times more effective, with much lesser side effects (in the form of
perioral or distal paresthesias)
 Dosage: up to 100 mg / day orally in 4 or 5 divided doses.
 Plasma Exchange (PLEX) or IV Immunoglobulin (IVIG) {for rapid
transitory improvement through removal of the pathological antibodies
(anti-nerve terminal antibodies)}
 Immunosuppressants (for more sustained improvement)
 Prednisone (Hostacortin)
 Azathioprine (Imuran)/ or Cyclosporine (Sandimmun)
/ Hostacortin-H 5 mg Tab.
Or: Deltacortril 5 mg Tab.
(1.5 mg/kg wt as a single dose after breakfast every other day)
/ Imuran 50 mg Tab. (50 mg daily initially, gradually increased to 2.5 mg
per kg wt daily divided in 3 doses, to be taken after meals)
N.B.:
 CBC including platelet count & LFTs should be checked weekly for 8
weeks & monthly thereafter.
138
 Small-cell carcinoma require surgical removal / radiotherapy, & / or
cytotoxic drug therapy.
MUSCLE DISEASES
MUSCULAR DYSTROPHIES
Classification & Characteristics of the Muscular Dystrophies
Disorder
Inheritance
Duchenne
type
X-linked
recessive
Becker's
X-linked
recessive
Limbgirdle
(Erb's)
Autosomal
recessive
(may be
sporadic or
dominant)
Facioscapulohumeral
Autosomal
dominant
Distal
Autosomal
dominant
(may be
recessive)
Ocular
Autosomal
dominant
(may be
recessive)
Autosomal
dominant
Oculopharyngeal
Age at
Onset
(years)
1-5 yrs
Distribution
Pelvic, then shoulder
girdle; later, limb &
respiratory muscles
Pelvic, then shoulder
girdle
Rapid progression.
Death within about
15 years after onset.
5-25 yrs
Slow progression.
May have normal
life span.
10-30 yrs Pelvic or shoulder
Variable severity &
girdle initially, with
rate of progression.
later spread to the other Possible severe
disability in middle
life.
Any age Face & shoulder girdle Slow progression.
initially; later, pelvic
Minor disability.
girdle & legs
Usually normal life
span.
40-60 yrs Onset distally in
Slow progression.
extremities; proximal
involvement later
Any age External ocular
muscles; may also be
(usually
mild weakness of face,
5-30)
neck, & arms
Any age As in the ocular form
but with dysphagia
DUCHENNE MUSCULAR DYSTROPHY



Prognosis
Inheritance: Sex-linked recessive
Sex & Age: Boys in the first decade
Onset & Course: Gradual, progressive
139

Starts by proximal pelvic girdle weakness, pseudohypertrophy of gastrocnemius muscle, waddling gait, positive "Gower's maneuver" when
arising from the floor (climbs upon his own body), elevated CPK,
contractures & skeletal deformities, shoulder girdle weakness occurs
after few years, wasting of thigh & arm muscles, with proximal
hypotonia & hyporeflexia, & intact sensations. Cardiomyopathy may
occur. EMG & muscle biopsy confirm the diagnosis.
 Duchenne muscular dystrophy (DMD) is not treatable.
 Ttreating DMD with steroids should begin in mid-childhood & continue
until adolescence or longer.
 DMD is a genetic disorder of the sarcolemma that causes progressive
weakness & death by the time the patient reaches his early 20s.
 Both skeletal & cardiac muscles degenerate, & are replaced with scar
tissue, but smooth muscle & the brain are affected to a lesser extent.
 The cause is a mutation in the dystrophin gene on the X chromosome
that results in loss or dysfunction of dystrophin protein, an important
part of the cell membrane structure.
D.D.: Becker's Muscular Dystrophy
It is a more benign form of sex-linked inheritance, & later age of onset.
Treatment:
Diet & Lifestyle:
 Weight control.
 Prophylaxis for pneumonia:
 Annual flu vaccine.
 Pneumovax every 5 years.
 Eliminate smoking.
Drug Treatment:
/ Prednisone 0.75 mg/kg per day.
Or: Deflazacort in the same dose as that of prednisone.
Interventional Procedures:
 Psychometric testing around the age of 5 yrs
 Electrocardiogram & echogram
 Sleep oximetry screening
 Nutritional assessment, feeding evaluation & modified video swallow
study
 Vocational rehabilitation evaluation
Surgery: Scoliosis repair
Assisstive Devices:
 Pulmonary rehabilitation devices
140

Assisstive cough therapy, human therapy, mechanical therapy, chest
percussion therapy, & intermittent positive pressure breathing
 Other devices:
 Wheelchair (manual & electric)
Physical Therapy & Exercise
Emerging Therapies:
 Gene therapy for DMD:
 Adeno-associated virus vector & mini-gene detector (a portion of
the dystrophin gene)
 Creatine, glutamine, & gentamycin may → Dystrophin production.
LIMB GIRDLE MUSCULAR DYSTROPHY

It is a hereditary disorder characterized by gradually progressive
shoulder & pelvic muscle weakness, wasting, hypotonia, & hypo-reflexia
with intact sensations.
 Serum CPK is elevated.
 It should be differentiated from treatable acquired conditions e.g.
polymyositis by EMG & muscle biopsy.
Treatment:
 No specific & wheelchairs may be required in advanced cases
 Surgical correction of kyphoscoliosis is rarely needed.
FACIOSCAPULOHUMERAL MUSCULAR DYSTROPHY

It is an autosomal dominant disorder, manifested in childhood, or in
second or third decades of life, & characterized by facial diplegia with a
characteristic transverse smile, bilateral gradual progressive weakness
of arms & legs. & shoulder girdle with scapular winging.
 Serum CPK is minimally elevated. EMG & muscle biopsy are helpful in
diagnosis.
Treatment: No specific treatment
 Crutches or a tripod cane may help in walking
 Molded leg braces may be needed
 Wheelchair in advanced cases.
MYOTONIAS
141
Channelopathies affecting the Cl&Na channels without periodic paralysis
Clinical
features
Inheritance
Gene defect
Age of
onset
Myotonia
congenita of
Thomsen
Dominant
Chromosome7
affects:
skeletal muscle
chloride
channel
Infancy to early
childhood
Myopathy
Muscle
hypertrophy
frequent; no
myopathy
Myotonia
Generalized
stiffness,
especially after
rest; improves
with exercise;
prominent
myotonia of eye
closure, but not
paradoxical
myotonia
Provocative
stimuli
Prolonged rest
or maintenance
of the same
posture
Exercise;
antimyotonia
therapy (e.g.,
mexiletine);
Achilles tendon
stretching helps
prevent need for
heel cordlengthening
surgery
Therapy
for
symptoms
Recessive
generalized
Myotonia
Recessive
Chromosome7
affects:
skeletal muscle
chloride channel
Acetazolamide
responsive Na
channel
myotonia
Dominant
Chromosome17
affects:
skeletal muscle
sodium channel
Myotonia
fluctuans
Dominant
Chromosome17
affects:
skeletal muscle
sodium channel
Late childhood,
occasionally starts
earlier or begins in
teens
Occasional muscle
wasting & weakness can occur late;
hypertrophy of
muscles frequently
occurs in legs
Generalized
stiffness, esp. after
rest; transient
weakness if
prominent after
complete relaxation for several
min; myotonia
occurs in eyes; no
paradoxical
myotonia occurs
First decade
First or second
decade
Rare
Rare; muscle
hypertrophy
common
Face, paraspinal
muscles,
paradoxical
myotonia of
eyelids, grip
limbs; varies in
severity, &
often there is
pain with
myotonia
Myotonia
Prolonged rest or
maintenance of
the same posture
Fasting, cold,
oral potassium,
infection
Exercise-restexercise, oral
potassium
Exercise; esp.
avoiding
prolonged rest;
antimyotonia
therapy (e.g.
mexiletine);
transient weakness
does not improve
after mexiletine
Acetazolamide,
mexiletine
monitoring
during & after
surgery for
rigidity &
rhabdomyolysis
Mexiletine;
avoid highpotassium diet;
monitor during
& after surgery
for rigidity &
rhabdomyolysis
MYOTONIC DYSTROPHY
142
Myotonic dystrophy is an autosomal dominant disorder characterized by
distal wasting & weakness in the hands & feet, associated with myotonia
(painless tightening of skeletal muscles after voluntary contraction,
percussion, or electrical stimulation). Myotonia is worsened by cold &
inactivity, & improved by warmth & exercises. Facial diplegia, cataracts,
mental retardation, frontal baldness, & cardiac arrhythmias are frequent.
EMG confirms the diagnosis. Silt-lamp examinations disclose cataracts. ECG
is also indicated.
MYOTONIA CONGENITA
It is an autosomal dominant disorder characterized by myotonia (voluntary,
mechanical, & electrical), & pseudohypertrophied muscles giving the patient
an athletic physique. No trophic or dystrophic manifestations.
Treatment
Diet & Lifestyle
 Avoidance of prolonged inactivity.
 Warm-up contractions of muscles are helpful to prevent rest-associated
myotonia
 Myotonia fluctuans has the distinct feature of exercise-induced delayedonset myotonia
 Intermittent carbohydrate snacks reduce serum potassium.
 Low-intensity exercise is often helpful in aborting or reducing the
intensity of hyperkalemic &, occasionally, hypokalemic attacks of
periodic paralysis.
 Patients with calcium channelopathy causing hypokalemic periodic
paralysis need to avoid high-carbohydrate diets.
 Low sodium intake is useful to prevent attacks of hypokalemic weakness.
 Patients with paramyotonia congenita, a sodium channelopathy, need to
avoid exposure to cold.
Drug Treatment
 For chloride & sodium channel myotonia & paramyotonia congenita:
/ Mexiletine (Mexitil)(150-200 mg bid-tid daily)
Or: Tocainide (Tonocard): (200 to 400 mg bid-tid)
 Mexiletine is superior to phenytoin & is similar in efficacy to tocainide
 For chloride channel & sodium channel myotonia & paramyotonia
congenita:
/ Acetazolamide (Diamox, Cidamex) (250 mg bid- tid).
Or: Dichlorphenamide (Oratrol) (50 mg bid, morning & evening)
143


Prevent attacks of hypokalemic periodic paralysis,
In hypokalemic periodic paralysis:
/ Hydrochlorothiazide (Hydrex) (12.5 to 50 mg/d)
 In hyperkalemic periodic paralysis:
/ Triamterene (Thiametrene): (50 to 100 mg/d. occasionally, 25 mg/d)
 In hypokalemic periodic paralysis that is made worse by treatment with
acetazolamide or another carbonic anhydrase inhibitor:
/ Spironolactone (Aldactone), (1 tab bid-tid)
 In hypokalemic periodic paralysis which become worse after treatment
with acetazolamide:
/ Propranolol (Inderal): (20 to 40mg given twice a day)
 In hypokalemic thyrotoxic periodic paralysis:
/ Potassium chloride
 Acute attack:
(25 mEq of potassium chloride given orally at 30-minute intervals.)
(Potassium should be given in 5% mannitol as a bolus of 0.05 to 0.1
mEq/kg body weight.)
 Preventive therapy:
(20 to 40 mEq of slow-release potassium chloride) (Slow-K) at bedtime.
PERIODIC PARALYSIS
Periodic paralyses comprise a group of autosomal dominant disorders, of
which the following are most important.
(1) Familial hypoklemic periodic paralysis
(2) Thyrotoxic hypokalemaic periodic paralysis
(3) Familial hyperkalemic periodic paralysis
(4) Paralysis periodica paramyotonia with hyperkalemia.
Familial Hypokalemic Periodic Paralysis
 An autosomal dominant trait, male: female = 3:1 between 15- 20 yrs
 Attacks of sudden flaccid weakness of 4 limbs, may be asymmetric, ē
areflexia, often occur in the morning, after heavy carbohydrate meal or
vigorous exercise followed by rest
 Serum K+: 2 – 3 mEq/liter
 EMG shows decreased muscle excitability
 Hyperaldosteronism & thyrotoxicosis may be associated ē episodic
weakness & hypokalemia
144
Treatment of Acute Attack:
 Potassium, either PO (10 –15 g of KCl in fluid), or IV KCl (40 – 60 mEq
in 500ml of 5% D/W infused over several hrs.
Prophylaxis:
 High – K, low – Na diet
 Spironolactone (Aldactone),100 mg PO daily or bid
 Thiamine HCl (Vit.B1), 50 – 100 mg daily
 Treatment of hyperthyroidism
 Acetazolamide (Cidamex), 250 – 500 mg PO q4 –6 hrs, to cause mildld
metabolic acidosis)
N.B.:
1. Patients with familial hypokalemic periodic paralysis are advised to
avoid precipitants of acute attacks e.g. high carbohydrate intake,
fatiguing exercises, & exposure to cold.
2. When such patients are subjected to surgery: use normal saline instead of
D5/W; warm the patient & the IV solutions; & avoid neuromuscular
blockers.
3. Chronic use of oral KCl does not prevent attacks of weakness or
paralysis.
Familial Hyperkalemic Periodic Paralysis
 An autosomal dominant trait, male = female, occurs in childhood.
 Attacks are milder & shorter (30 – 90 min) than in hypokalemic type
 Resp. ms are spared, but trunk & prox. limb ms are involved.
 Mild myotonia may be present.
 EMG shows hyperirritability between attacks, & myotonic-like discharges
during attacks
Treatment of Acute Attack:
 IV infusion of 10% Ca gluconate, 10 – 20 ml
Prophylaxis:
 Acetazolamide (Cidamex)250 mg PO qid
 Hydrochlorothiazide (Esidrex), 50 – 100 mg PO daily
N.B.:
(1) A high carbohydrate diet is beneficial
(2) Avoidance of exposure to cold & overexertion is helpful.
(3) IV Calcium Gluconate 10% Amp. may abort an acute attack.
Thyrotoxic Hypokalemic Periodic Paralysis
A. Treatment of Acute Attacks:
Same drug regimen as hypokalemic periodic paralysis
145
B. Preventive Therapy:
• Control of hyperthyroidism
/ Inderal 40 mg Tab. (20-40 mg qid)
Or: Aldactone-A 25 mg Tab.
(25 mg bid, gradually ↑ up to 100 mg daily)
N.B.:
(1) Acetazolamide (Cidamex)is contraindicated.
(2) Precipitants are the same as for the familial form.
Paramyotonia Congenita of Eulenburg
• Spontaneous attacks of weakness unrelated to cold + hypokalemia.
• Treated by a potassium-sparing diuretic e.g.:
/ Aldactone-A 25 mg Tab.
(1 tab bid-tid)
• Acetazolamide (Cidamex) & thiazides (e.g. Hydrex) are contraindicated.
Paralysis Periodica Paramyotonia with Hypokalemia
/ Hydrex 25 mg Tab. ( Adults: 1 tab./d.Children: 2 mg/kg/d in 2 doses).
POLYMYOSITIS
Clinically:
 It is an inflammatory autoimmune muscle disease
 Acute or subacute, or chronic onset
 Proximal muscle weakness + involvement of pharyngeal muscles
(dysphagia) & posterior neck muscles (forward drooping of head), but
sparing facial muscles.
 Skin rash on dorsum of hands, proximal digits, knees, or elbows
 Cardiac involvement in 30% of pts
 Pts with polymyositis have a 20% incidence of cancer
Investigations:
 Muscle enzymes (CK, transaminase, & aldolase) are elevated
 ESR is raised
 EMG is non-specific, but shows a combination of myopathic pattern
(brief, small-amplitude polyphasic motor units), & neuropathic pattern
(positive sharp waves, fibrillations, & high-frequency repetitive
discharges)
 Muscle biopsy: shows an inflammatory infiltrate & necrosis of muscle
fibers in 85-90% of pts
146
Treatment:
1. Corticosteroids: accelerate remission & ↓ morbidity
 Usually used in combination ē either methotrexate or azathioprine
 Prednisone, (Hostacortin) 60-100 mg PO every morning, gradually
↓ with improvement to 40 mg & maintained for several months.
 The rate of dosage reduction is 5-10 mg every 3-7 days
 In 6-12 mns most pts can be kept on 15-20mg every morning, or 3040mg every other day.
 Response to therapy & relapse can be monitored by muscle enzyme
determination
 Attempting to D/C steroids should be made after 2 yrs of therapy
Steroid myopathy may occur (more in females), & calls for dose
reduction
2. Immunotherapy: May be of benefit in chronic progressive cases.
 Immunosuppressive drugs may be effective if used alone or
combined with smaller dosage of prednisone
/ Methotrexate (IV over 20-60 min, starting at 0.4 mg/kg/treatment, & ↑ to
max. of 0.8 mg/kg/treatment in 2-3 wks. Given weekly until improvement
occurs, reduce to biweekly, then triweekly, then monthly doses are given
for 10-24 mns)
 Stomatitis & GI symptoms call for ↓ of dosage
 Leukopenia & hepatic toxicity require discontinuation
Or: Azathioprine (Imuran), (1.5-2 mg/kg PO, either alone or with
prednisone. Dosage is gradually. ↑ until the WBC count ↓ & that
dosage is continued until remission of polymyositis).
 Bone marrow suppression, anorexia, nausea, vomiting, & jaundice
are the major side effects
3. Other therapeutic measures:
 Bed rest in acute cases
 Physical therapy on improving
 Braces & other physical measures for chronic weakness
DEMYELINATING DISEASES
MULTIPLE SCLEROSIS (M.S.)




Insidious onset & fluctuant course with remissions & relapses.
Affects both sexes, females more than males.
Age of onset: 15-60 years
Manifestations:
147





Motor: incoordination, weakness, spasticity.
General sensory: radicular pain, paresthesias
Visual: blurring of vision, diplopia
Proprioception: sensory ataxia.
Cranial nerves: Optic atrophy (pallor of temporal halves of optic
discs); Oculomotor (diplopia); Trigeminal (loss of corneal reflex,
hemifacial pain); Facial palsy; Vestibular (vertigo); Bulbar
(pseudo-bulbar palsy)
 Ataxic gait
 Bladder & bowel dysfunction + Impotence.
 CSF: increased immunoglobulin G (IgG), the presence more than 2
oligoclonal bands & elevated myelin basic protein.
 CT scan & MRI of the brain disclose the sclerotic plaques.
 Visual evoked potentials (VEPs) & Brainstem auditory evoked potentials
(BAEPs) are helpful in diagnosis.
Treatment:
1. Symptomatic Treatment
2. Immunotherapy
3. Aerobic Exercise Program, 3 times weekly
4. Neurorehabilitation
Symptomatic Treatment in MS
Chronic symptom
Medication/Management
Spasticity
/ Baclofen (Lioresal), oral 30-100 mg/day, intrathecal up to
200 mg/day; most useful available medication
Or: Tizanidine (Sirdalud), 6-24 mg/day
Or: Dantrolene (Dantrium), 100-400 mg/day
Or: Diazepam (Valium), 2-30 mg/day
/ Botulinum toxin (Botox A), (selective cases)
 Physiotherapy
Fatigue
/ Amantadine (Adamine), 200 mg/day, not to be
taken in the evening
/ Pemoline (Cylert), 37.5 mg/day
/ 4- Aminopyridine (Fampridine), 10 mg, bid-tid
 Selective serotonin reuptake inhibitors (SSRIs),
e.g.
/ Fluoxetine (Prozac), 20-40 mg/day
148
MS-related acute pain
/ Carbamazepine (Tegretol), 100-200 mg bid-tid
/ Benzodiazepines e.g. Clonazepam (Rivotril) 0.52mg
/ Baclofen (Lioresal), 10-25 mg tid
MS-related chronic
pain
/ Amitryptiline (Tryptizol), 25-50 mg tid
/ Carbamazepine (Tegretol), 100-200 mg tid
/ Baclofen (Lioresal), 10-25 mg tid


Physiotherapy
Occasional surgical procedures
Musculoskeletal pain
 Nonsteroidal anti-inflammatory drugs (NSAIDs)
Pain due to
paroxysmal symptoms
/ Carbamazepine (Tegretol) 100 – 200 mg tid
/ Gabapentin (Neurontin) 300 mg tid
Pain due to spasticity
 See spasticity
Bladder dysfunction
/ Anticholinergics, e.g. Oxybutynin (Uripan), 5 mg
Tremors
Sexual dysfunction
tid
/ Desmopressin (Minirin) 0.1 mg & 0.2 mg Tab, 1-2
tabs daily at bedtime
 α- Blockers, e.g. Prazosin (Minipress)
 Bethanechol
 Clean intermittent self- catheterization (CISC)
 No effective long-term treatment is available, the
following have been tried for long-term relief:
 Medical: Carbamazepine (Tegretol), Clonazepam
(Rivotril), Primidone (Mysoline), Propranolol
(Inderal), Clozapine (Leponex),
 Surgical: Stereotactic surgery
 Physical: Weight application
/ Papaverine (Vasorin) (intracorporeal injection)
Or: Prostaglandin E (Caverject) (intracorporeal inj.)
Or: Sildenafil (Viagra), PO 50 – 100 mg prn
 Lubricants
 Devices e.g. Penile prosthesis
149
Vertigo
/ Ondansetron (Zofran), 4 mg & 8 mg Tabs, 1 tab
bid.
Temperature &
exercise sensitivity &
weakness
/ 4-Aminopyridine (Fampridine), 10 mg bid-tid
Depression

Antidepressants (SSRIs or TCAs)
Forced pathological
crying & emotional
instability

SSRIs
e.g. Fluoxetine (Prozac), 20-40 mg /d
Seizures & other
paroxysmal symptoms
 Anticonvulsants (AEDs)
Immunotherapy of MS
1. Glucocorticoids & ACTH: for treatment of MS relapses
a) IV Methylprednisolone, (Solu-Medrol) (500-1000 mg/d. 3-5 days)
b) Oral prednisone, e.g. Hostacortin or Deltacortril 5 mg Tabs.
(1 mg/kg/d)
Or: ACTH 20 or 40 Units.
Or: Synacthen-Depot Amp.
2. Conventional Immunosuppressive Treatments:
a) Azathioprine (Imuran), for RRMS, Oral 50 mg tid
b) Cyclophosphamide (Cytoxan, Endoxan), for CPMS, IV 400-500 mg
daily for 2 wks
c) Cyclosporin (Sandimmun), 5 mg /kg/d, in two divided doses
d) Methotrexate (Methotrexate)
3. Immunomodulators:
 Interferon- β (IFN β):
 Interferon b-1b (Betaseron, Betaferon)
- Low (6 MIU) & High (8 MIU), SC/other day
 Interferon b-1a (Avonex), 6 MIU IM every wk.
 Interferon b-1a (Rebif):
- Low (6MIU) (SC 3 times/wk)
- High (12MIU) (SC 3 times/wk)
 Copolymer-1 (Copaxone),( 20 mg SC daily)
4. Antiviral agents e.g. Acyclovir (Zovirax) PO 800 mg tid +Other drugs:
/ IV Immunoglobulin (IVIg): (0.2 gm/kg body wt once/month)
150
/ Linomide (quinoline-3-carboxamide), (PO 2.5 mg daily)
/ Cladribine (Leustatin), (0.07 mg/kg s.c. 5days each month for 6 months)
/ Mitoxantrone (Novantrone)
(12 mg/m2 or 5 mg/m2 IV every 3 months for 2 yrs)
OPTIC NEURITIS
Clinical Features
 Either papillitis or retrobulbar neuritis
 Abrupt loss of vision over 2-3 days
 Central scotoma
 Pain in the affected eye in 70% of cases
 Loss of colour vision (achromatopsia)
Investigations
 MRI of brain & orbits ê gadolinium enhancement
 CSF examination may reveal pleocytosis & ↑IgG
 VEPs are abnormal
 Mapping of visual field reveals central scotoma
Relation to MS
 40% of MS pts have at least one attack of optic neuritis
 The risk of MS in pts with one attack of optic neuritis is 34% for males &
74% for females
 Optic neuritis is the first symptom in 10 – 15% of MS pts.
Prognosis & Treatment
 50 % of pts have recovery of vision within 1month & 75% within 6
months
 IV methylprednisolone (Solu-Medrol), 250 mg q6 hrs for 3days, followed
by:
 Oral prednisone,(Hostacortin or Deltacortril 5 mg tabs) 1 mg/kg for 11
days
NEUROMYELITIS OPTICA
(DEVIC'S DISEASE)
It has been regarded as a form of M.S. (D.S.) & is characterized by the
occurrence of optic neuritis or retrobulbar neuritis in one or both eyes
(central scotoma, pain in the eye & visual deterioration up to blindness) +
transverse myelitis (acute paraplegia or quadriplegia). Optic neuritis may
occur alone, acute transverse myelitis may develop without optic neuritis, or
both may co-exist.
Treatment: same regimen as in MS.
151
ACUTE DISSEMINATED ENCEPHALOMYELITIS (D.E.M.)
Two Types:
I. Postinfectious encephalomyelitis:
Develop 4-6 days after viral exanthema of measles, chickenpox,
smallpox, or rubella
II. Postvaccinal encephalomyelitis:
Develop 10-15 days after vaccination against smallpox or rabies
Clinical Features
 Mild cases: → headache, stiff-neck, fever, & confusion
 Severe cases: → convulsions, quadriplegia, cerebellar ataxia, multiple
cr.n. palsies, sensory loss, & coma
Investigations
 CSF: → changes consistent ê aseptic meningitis
 MRI Brain: → widespread white matter lesions
Prognosis:
 Mortality is 10-20% in post-measles type, 30-58 % in post-vaccination.
 25-50% of survivors have severe neurologic sequelae
Treatment:
 IV Solu-Medrol, 500 mg/day q 12 hrs for 3 days, followed by:
 Prednisone PO, 60-80 mg/day for 7 days, followed by a taper of 20 mg
each week
 Anticonvulsants for seizures
 Mannitol 20% for cerebral edema
 Supportive measures
ACUTE TRANSVERSE MYELITIS
 A common complication of MS, or
 An idiopathic illness, or
 Postvaccinal, or
 Postinfectious, or
 A complication of HIV infection, S.L.E., Syphilis, or Borrelia
burgdorferi infection
Criteria of Diagnosis:
1. Acute or subacute febrile onset with back pain.
2. Flaccid paraplegia + Hyporeflexia or areflexia + Lost abdominal reflexes
below the level of the lesion + absent plantar responses.
3. Sensory loss below the level of the lesion.
4. Retention of urine & faeces at first & precipitency of micturition later on.
5. Bed-sores are frequent.
152
6. Spasticity, Hyperreflexia & Extensor plantar responses occur after
recovery from the shock stage.
7. MRI with gadolinium enhancement (for brain & spinal cord) may reveal
MS plaques.
8. CSF examination: CSF profile is similar in myelitis due to MS &
idiopathic myelitis
Prognosis:
 Idiopathic, postvaccinal, & postinfectious types are monophasic selflimited illnesses. Recovery often occurs.
Treatment:
 Treatment of the cause e.g. infection (e.g. HIV, syphilis, B. burgdorferi),
or S.L.E.
 If due to MS, or is idiopathic, postvaccinal, or postinfectious:
/ IV methylprednisolone, (Solu-Medrol)
(500-1000 mg i.v. infusion over 1 hr daily for 3-5 days,) followed by;
/ Oral prednisone (Hostacortin, Deltacortiril) (as if it was an attack of MS).
153
NEURODEGENERATIVE DISEASES
AIZHEIMER'S DISEASE (AD)
Diagnostic Criteria for Dementia of the Alzheimer’s Type
Multiple cognitive deficits
 In memory
 One or more of language, praxis, gnosis, executive functioning
Causing
 Significant impairment & decline in social or occupational functioning
 Gradual onset & continuing cognitive decline
Not due to
 Other CNS or substance-induced conditions
 Deficits not exclusively during course of delirium & not better accounted
for by depression or schizophrenia
Clinical Stages Typical of Alzheimer’s Disease
Stage I (1 – 3 years)

Memory

Visuospatial skills







Language
Personality
Psychiatric features
Motor system
EEG
CT/MRI
PET/SPECT
Stage II (2 – 10 years)

Memory

Visuospatial skills

Language

Calculation

Praxis

Personality

Psychiatric features

Motor system

EEG

CT/MRI
New learning defective, remote recall mildly impaired
Topographic disorientation, poor complex
constructions
Poor word list generation, anomia
Indifference, occasional irritability
Sadness or delusions in some
Normal
Normal
Normal
Bilateral posterior parietal hypometabolism /
hypoperfusion
Recent & remote recall more severely impaired
Poor constructions, spatial disorientation
Fluent aphasia
Acalculia
Ideomotor apraxia
Indifference or irritability
Delusions in some
Restlessness, pacing
Slowing of background rhythm
Normal or ventricular dilatation & sulcal enlargement
154

PET/SPECT
Stage III (8-12 years)

Intellectual
functions

Motor

Sphincter control

EEG

CT/MRI

PET/SPECT
Bilateral parietal & frontal hypometabolism /
hypoperfusion
Severely deteriorated
Limb rigidity & flexion posture
Urinary & fecal incontinence
Diffusely slow
Ventricular dilatation & sulcal enlargement
Bilateral parietal & frontal
hypometabolism/hypoperfusion
D.D. of AD:
1. Vascular dementia (Multi – infarct dementia)
2. Parkinson’s disease (PD)
3. Fronto – temporal dementia (FTD)
4. Depressive pseudodementia
5. Normal pressure hydrocephalus (NPH)
6. Senile Dementia with Lewy – Bodies (DLB)
7. Focal brain atrophy
8. Hypothyroidism (Myxedema)
Methods for Clinical Diagnosis of AD:
 Clinical history
 Physical examination
 Mini mental state examination (MMSE)
 Blood chemistry e.g. B12, thyroxin
 Neuropsychological testing e.g. Neuropsychiatric Inventory (NPI)
 Brain CT/MRI scans
 Functional imaging (PET &/or SPECT)
 CSF analysis
 Brain biopsy
Treatment:
The Cholinergic HypothesisSymptomatic treatment of AD is currently based
on the cholinergic hypothesis, which states that many of the cognitive,
functional, & behavioral symptoms derive from a reduction in brain
acetylcholine activity secondary to the loss of cholinergic neurons in the
nucleus basalis of Maynert & other nuclei projecting to the hippocampus &
mesial temporal region
155
Summary of Drug Therapies in AD
Drug
Cholinergic Agonists

Tacrine

Donepezil

Rivastigmine

Galantamine
Antipsychotic Drugs

Haloperidol

Risperidone

Olanzepine

Clozapine

Thioridazine
Antidepressants

Fluoxetine

Paroxetine

Sertraline

Venlafaxine

Nortriptyline

Trazodone
Benzodiazepines

Diazepam

Clonazepam

Lorazepam
Indications
Treatment of
noncognitive behavioral
manifestations
Precautions
Trials to date are based on
relatively small populations;
large, placebo - or standardcontrolled trials are needed
Treatment of disturbing
hallucinations or frank
persecutory delusions
Treatment must be actively
monitored for effectiveness
& need to continue; side
effects & drug interactions
are key factors determining
drug selection
Serotonergic drugs may
become first-line treatments
for depression, aggression,
agitation, & disturbed sleep,
but controlled trials are
needed to justify their use
AEDs & Mood Stabilizers

Carbamazepine

Valproic acid
Promising alternatives
for treatment of
agitation
Treatment of significant
dysphoria; serotonergic
drugs are often useful
after trials of
antipsychotic drugs
have failed to improve
behavioral disturbances
Short-term use to help
tide patients over
difficult adjustment
periods
Many potential risks for
elderly patients with modest
beneficial effects; should
not be used as a first-line
drug for treatment of
behavioral symptoms
Used clinically despite
limited published
experience
Therapeutic doses of cholinesterase inhibitors in AD
Tacrine
(Cognex)
80-120 mg/day (qid)
Donepezil
(Aricept)
5-10 mg/day (o.d)
Rivastigmine (Exelon)
6-12 mg/day (bid)
Galantamine (Reminyl)
16-24 mg/day (bid)
Non-Cognitive Pharmacological Treatment of AD
Depression
/ Fluoxetine (Prozac),
Or: Paroxetine (Seroxat),
Or: Sertraline (Lustral),
Or: Venlafaxine (Effexor),
10 – 40 mg / d
10 – 40 mg / d
50 – 200 mg / d
75 – 375 mg / d
156
Or: Nortriptyline (Aventyl),
Anxiety
/ Lorazepam (Ativan),
Or: Alprazolam (Xanax),
Or: Buspirone (Buspar),
Agitation
/ Trazodone (Trittico),
Or: Haloperidol (Haldol, Safinace),
Or: Risperidone (Risperdal),
Or: Thioridazine (Melleril),
Delusions
/ Risperidone (Risperdal),
Or: Haloperidol (Haldol, Safinace),
Or: Thioridazine (Melleril),
Or: Olanzepine (Zyprexia),
Or: Clozapine (Leponex),
Insomnia
/ Warm milk
Or: Trazodone (Trittico),
Or: Diphenhydramine (Benadryl),
Or: Zolpidem (Stilnox) ,
Or: Short-acting benzodiazepines
50 – 150 mg / d
0.5 – 1 mg tid
0.25 – 1.25 mg tid
5 – 10 mg tid
50 – 100 mg / d
0.5 – 3 mg / d
0.5 – 3 mg / d
30 – 60 mg
0.5 – 3 mg / d
0.5 – 3 mg / d
30 – 60 mg / d
2.5 – 15 mg / d
6.25 – 50 mg / d
50 – 100 mg / d
25 – 50 mg / d
5 – 10 mg / d
Cognitive Enhancers
Most Common:
/ Ginkgo biloba (Tebonina),
Or: Piracetam (Nootropil),
Or: Pyritinol (Encephabol, Cerebrotone),
Or: Meclofenoxate (Lucidril),
Or: Co-Dergocrine Mesylate (Hydergine),
Or: Piribedil (Trivastal),
Less Common:
/ Nicergoline (Sermion),
Or: Cinnarizine (Stugeron),
Or: Naftidrofuryl (Praxilene),
Or: Pentoxifyline (Trental),
40 mg tid
800 mg tid
200 mg tid
500 mg bid
1.5 mg bid
50 mg bid
5 – 10 mg tid
25 mg tid
200 mg bid
400 mg bid
MOTOR NEURONE DISEASE
AMYOTROPHIC LATERAL SCLEROSIS (ALS)
Aetiology:
1. Age: Late middle life.
2. Sex: Males more tham females (2:1).
3. Commonly sporadic. .
4. May be inflammatory, toxic, or traumatic.
157
Pathology:
* Degeneration of ganglion cells & Betz cells in motor cortex.
* Degeneration of pyramidal fibres in the internal capsule.
* Degeneration of the pyramids &/or the medullary motor nuclei of cranial
nerves.
* Degeneration of pyramidal tracts in lateral columns of the cord.
* Degeneration of A.H.Cs.
* Degeneration of ventral roots with atrophy of denervated muscles.
Presenting complaints in patients with suspected ALS
 Muscle weakness (usually focal but may be multifocal)
 Bulbar symptoms (dysarthria, dysphagia, drooling)
 Spinal symptoms (weakness in an arm or leg)
 Respiratory symptoms (exertional dyspnea)
 Fatigue
 Weight loss
Diagnostic Criteria for ALS
The diagnosis of ALS requires the presence of:
 Signs of lower motor neuron (LMN) degeneration by clinical,
electrophysiologic, or neuropathologic examination
 Signs of upper motor neuron (UMN) degeneration by clinical
examination
 Progressive spread of signs within a region or to other regions with the
absence of:
 Electrophysiologic evidence of other disease processes that might
explain the signs of LMN &/or UMN degeneration
 Neuroimaging evidence of other disease processes that might
explain the observed clinical & electrophysiologic signs
Diseases Mistaken for ALS
 Benign fasciculations
 Parkinson's disease (PD)
 Kennedy's disease (X-linked bulbospinal muscle atrophy)
 Brainstem stroke
 Lumbosacral stenosis
 Cervical myelopathy
 Carpal tunnel syndrome (CTS)
 Brachial plexopathy
 Neuropathy
158
The diagnosis of ALS is supported by the following EMG findings:
 Reduced recruitment (firing rate > 10 Hz)
 Large motor unit action potentials
 Fibrillation potentials
 Unstable, large motor unit action potentials
 Reduced motor unit action potentials
 Reduced motor unit number estimates (MUNE) & increased macroEMG unit action potentials
Other Diagnostic Tests Performed in ALS
The following diagnostic tests are not mandatory but can be used primarily
to rule out other similar diseases, & secondarily as confirmatory tests
 Neuroimaging
 Ascertain findings that may exclude other disease processes
 Muscle biopsy
 Ascertain acute & chronic denervation
 Identify LMN degeneration in clinically uninvolved regions
 Laboratory tests
 CBC & serum chemistry panel
 Endocrine function tests (especially thyroid function tests)
 Heavy metals (do not perform unless there has been a definite
history of exposure)
 GM1 antibodies (helpful when positive if patient has multifocal
motor neuropathy)
Drug Treatment:
/ Riluzole (Rilutek), (50 mg bid)
 Riluzole is a benzothiazole derivative with complex effects on glutamate
neurotransmission including inhibition of presynaptic glutamate release
Symptomatic Treatment of ALS
Symptoms
Weakness /
ambulation
Exercise
Activities of daily
living (ADL)
Treatment
Physical therapy, orthopedic & assistive devices, canes,
walkers, specialized wheelchairs
Exercise in grade 4 or 5 muscles only
Safety devices for bath & toilet, dressing & feeding
Assistive devices
159
Cramping
Spasticity
Pain
Dysphagia /
swallowing / weight
loss
Salivation
Loss of speaking
Quinine (Lioresal),diazepam (Valium), phenytoin
(Epanutin)
Baclofen
Analgesics, opiates,
Transcutaneous electrical nerve stimulation (TENS)
Blenderized food, supplements, feeding tube,
percutaneous gastrostomy (PEG)
Suction machines; Amitriptyline (Tryptizol) or Atropine
Paper & pencil, specialized voice ability synthesizers,
Etran communications board, TDD (telecommunication
device for the deaf), microcomputer-based instruments
Depression / insomnia
Antidepressants (offer universally), anxiolytics,
electrically powered adjustable bed
Sleep disturbance
Clonazepam or Sinemet CR; ventilatory assistance
(BiPAP or nasal CPAP)
Respiratory/pulmonary
weakness
Antisecretory agents, cough medication, tracheostomy;
BiPAP, CPAP, chest cuirass volume ventilators
Infections
Antibiotics; prophylactic vaccines (pneumococcal,
influenza)
Hospice
Some Common Symptoms in ALS & Their Treatment
Symptoms
Cramps
Cause
? Changes in motor
neurone Na+ channel
function
Treatment
/ Quinine sulphate 200 mg bid
/ Carbamazepine (Tegretol) 200 mg tid
Or: Phenytoin (Epanutin) 100 mg tid
/ Magnesium
/ Verapamil (Isoptin) 40 – 80 mg tid
Spasticity
Corticospinal tract
damage
/ Baclofen (Lioresal)10-80mg daily
Pseudobulbar
syndrome
/ Amitriptyline (Tryptizol) 25-50 mg noct.
Emotional
Lability
Or: Tizanidine (Sirdalud) 6-24mg daily
Or: Dantrolene(Dantrium) 25-100mg /d
Or: Intrathecal baclofen (Lioresal)
Or: Memantine 10-60mg daily
Or: SSRIs (e.g., citalopram, fluvoxamine)
/ Citalopram (Cipram) 20 mg o. d.
/ Fluvoxamine (Faverin) 50 mg o. d.
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Sialorrhea
Bulbar weakness
/ Atropine eye drops sub-lingual
Or: Atropine (tabs/liquid)
0.25-0.75 mg tds
Or: Benztropine (Cogentin) (tabs/liquid)
Or: Benzhexol (tabs) 1-2 mg bid-tid
Or: Hyoscine (tabs/transdermal patches)
/ Amitriptyline (Tryptizol) (tabs/liquid)
/ Glycopyrrolate (liquid: s.c./ i.m. /via
PEG)
 Salivary gland irradiation
 Transtympanic neurectomy (?)
 Botox injection to salivary glands (?)
Agents Approved or Under Investigation for the Treatment of ALS
 Glutamate antagonists
 Riluzole (Rilutek) (approved for the treatment of ALS)
 Gabapentin (Neurontin) (commercially available for treatment of
seizure disorders & neuropathic pain)
 Neurotrophic factors
 Insulin-like growth factor-1 (IGF-1)
 Glial-derived neurotrophic factor (GDNF)
 Cardiotrophin-I (CT-1)
 Neurotrophin-3, neurotrophin-4 (NT-3, NT-4)
 Protease inhibitors
 1Antichymotrypsin (ACT)
 Protease-nexin-1 (PN-1)
 Antioxidants
 Vitamin E
NORMAL PRESSURE HYDROCEPHALUS (NPH)
Clinically: gait disturbance (ataxia) + dementia + incontinence + cerebral
ventricular enlargement with normal CSF pressure (communicating
ventriculomegaly).
 NPH should be differentiated from:
 Parkinson's disease
 Multi-infarct dementia
 Bifrontal space-occupying lesion (S.O.L.)
 Obstructive hydrocephalus e.g. in aqueduct stenosis
161


 Pernicious anemia
 Hypothyroidism
NPH improves with shunting procedures in most cases.
Diagnostic procedures include: CT scan or MRI of the brain, radioisotope cisternography, CSF pressure measurement & analysis.
Probable shunt- responsive NPH & Improbable shunt-responsive NPH
Clinical
Probable shuntresponsive NPH



Improbable shuntresponsive NPH



CT
Gait disturbance:
prominent or first sign
Mental impairment:
not present to
moderate
Urinary
incontinence: not
obligate


Absence of gait
disturbance
Urinary incontinence:
prominent or first sign
Dementia with
predominant cortical
dysfunction (e.g.,
aphasia or agnosia)
One of the following:

Only slightly dilated
ventricles

Marked cortical atrophy

Moderate or severe
white matter lesions
without ventricular
enlargement


Rounded frontal horns
Moderate or greater
ventriculomegaly
No cortical atrophy
No severe white
matter lesions
Possible shunt responsive NPH includes patients who cannot be classified
into either probable or improbable shunt-responsive NPH, e.g., those in
whom CT shows moderate or pronounced white matter disease, but
insufficient to explain severe ventriculomegaly & those with possible
combinations of two diseases, such as Binswanger’s or Alzheimer’s disease
with NPH.
Treatment:
 Ventriculoperitoneal shunt (+ Brain biopsy in selected cases with severe
dementia to confirm the diagnoses)
 Complications of surgery: e.g., subdural & intracerebral hematoma,
cerebral infarction, infection, seizures, shunt failure, pulmonary
embolism, & death.
162


Follow up CT scans should be done.
Ambulatory aids (e.g. crutches, walkers. & wheelchairs); physiotherapy;
supportive care; management of urinary incontinence & UT infections &
psychiatric treatment for agitation, or psychosis (Safinace 1.5 mg or 5
mg tid), or depression (Tryptizol 10 mg or 25 mg tid)
163
NUTRITIONAL DEFICIENCY DISORDERS
PELLAGRA
Criteria of Diagnosis:
1. Cutaneous lesions: [Dermatitis, erythema, desquamation & thickening of
the skin on the exposed parts of the body & over the bony prominences].
2. Castro-intestinal disturbances: e.g. glossitis, stomatitis & achylia gastrica
+ Diarrhea.
3. Neuropsychiatric disturbances:
i. Psychiatric: e.g. Depression, Dementia, Mania, amnesia, stupor or
coma.
ii. Neurological: e.g. Peripheral Neuropathy, Myelopathy (Lateral
sclerosis → Pyramidal affection; or Subacute Combined Syndrome →
Pyramidal + Posterior Column + Peripheral nerve affection) &
Encephalopathy.
BERIBERI
Criteria of Diagnosis:
1. Acute Polyneuritis.
2. Cardiac Manifestations: Heart failure, cardiac dilatation, dyspnea,
tachycardia, palpitation & oedema.
SUBACUTE COMBINED DEGENERATION OF THE CORD
Criteria of Diagnosis:
1. Nervous Symptoms:
i. Sensory: Glove & stocking superficial sensory loss + Impaired sense of
position, sense of movement & vibration sense + Tender calves
+ Sensory ataxia with positive Romberg's sign.
ii. Motor: [Weakness, spasticity, hyperreflcxia & extensor plantar
response].
iii. Sphincters: [Precipitency of micturition followed by retention or
incontinence + Impotence].
iv. ± Cranial Nerves: [Bilateral primary optic atrophy + Nystagmus].
v. ± Mental changes : [Dementia, Depression & Psychosis].
2. Gastro-intestinal disturbances: e.g. achylia gastrica, glossitis, dyspepsia
& diarrhea.
3. Addisonian (Macrocytic) Anaemia.
164
CNS NEOPLASMS
BRAIN TUMOURS
PATHOLOGICAL VARIETIES:
1. Gliomas: 40- 45%
i. Medulloblastoma.
ii. Glioblastoma Multiforme.
iii. Astrocytoma.
iv. Ependymoma.
v. Oligodendroglioma.
2. Meningiomas: 10%
3. Acoustic Neuroma: 5-10%.
4. Angiomas.
5. Craniopharyngiomas.
6. Pituitary Tumours:
i. Chromphobe adenoma,
ii. Acidophil adenoma.
iii. Basophil adenoma.
7. Pinealoma.
8. Colloid Cysts of the 3rd. Ventricle.
9. Metastatic Tumours: 15-20%.
10. Tumours of infective origin:
i. Tuberculoma.
ii. Gumma.
iii. Parasitic cysts,
iv. Brain abscess.
CLINICAL PICTURE:
A. General Symptoms of Increased ICT:
1. Headache.
2. Vomiting.
3. Papilloedema.
4. Convulsions.
5. Confusion or coma.
6. Hypopituitarism may occur.
7. Hypothalamic affection may occur (Somnolence, glycosuria,...etc).
8. Vertigo.
B. False Localizing Signs:
1. Cranial nerve palsies e.g. 6th & 3rd.
2. Bilateral extensor plantar responses.
165
3. Bilateral grasp reflexes.
4. Cerebellar symptoms in frontal lobe tumours.
5. Midbrain symptoms.
C. Focal Symptoms:
1. Frontal Lobe Tumours:
a) Prefrontal Tumours:
i. Headache.
ii. Mental symptoms e.g. dementia, incontinence of urine & stools.
iii. Convulsions.
iv. Catatonia.
v. Motor aphasia.
vi. Grasp reflex.
vii. Contralateral hemiparesis.
viii. Foster-Kennedy syndrome.
ix. Cerebellar symptoms.
b) Pre-central Tumours:
i. Focal Jacksonian fits (Pyramidal excitation).
ii. Monoplegia or paraplegia (Pyramidal destruction)
2. Temporal Lobe Tumours:
i. Uncinate fits.
ii. Partial impairment of taste & smell on the ipsilateral side.
iii. Partial impairment of hearing (bilateral), tinnitus or auditory
hallucinations.
iv. Major fits may occur.
v. Aphasia in left-sided lesions.
vi. Field defect e.g. crossed upper quadrantanopia.
vii. Pyramidal signs on the opposite side.
3. Parietal Lobe Tumours:
i. Irritative Lesion → Sensory Jacksonian fits.
ii. Destructive Lesion → Cortical sensory loss on the opposite side,
iii. Hypotonia & wasting.
iv. Field defect e.g. crossed homonymous lower quadrantanopia.
v. Speech disorders e.g. alexia, agraphia & acalculia in lesions of left
angular gyrus.
vi. Ideational apraxia in lesions of the left supramarginal gyrus.
4. Occipital Lobe Tumours:
i. Symptoms & signs of increased ICT are early e.g. headache.
ii. Convulsions with visual aura.
iii. Visual field defect e.g. crossed homonymous hemianopia.
166
iv. Visual agnosia in left-sided lesions.
v. Neighbourhood symptoms: e.g. alexia fc agraphia, auditory hallucinations, word-deafness, cortical sensory loss, weakness, cerebellar
signs (nystagmus, hypotonia,...etc.).
5. Corpus Callosal Tumours:
i. Mental symptoms e.g. apathy, drowsiness & dementia.
ii. Convulsions.
iii. Grasp reflex.
iv. Apraxia.
v. Double hemiplegia.
vi. Choreiform movements & tremors.
vii. Raised CSF proteins.
6. Cerebellar Tumours:
a) Midline Cerebellar Tumours e.g. Medulloblastoma:
* Children.
* Early signs of increased ICT.
* Tonic fits (midbrain compression).
* 6th & 7th nerve palsies
* No nystagmus.
* Weakness of the limbs may occur due to increased ICT.
b) Hemispherical Cerebellar Tumours e.g. Astrocytoma& Haemangioblastoma :
* Early signs of increased ICT.
* Nystagmus more on looking to the lesion.
* Ataxia of the limbs on the side of the lesion.
* Wide-based staggering gait.
* Hypotonia.
* Neighbourhood symptoms: e.g. 5,6 & 7th nerve palsies,pyramidal
signs on the opposite side, sensory loss on the opposite side. 7.
7. Acoustic Neuroma: (A cerebellopontine angle tumour).
i. Tinnitus, Vertigo & Deafness (progressive).
ii. Headache, but papilloedema & vomiting are late.
iii. Ipsilateral 5, 6 & 7th nerve palsies.
iv. Ipsilateral cerebellar ataxia.
v. Contralateral hemiparesis & hemianesthesia may occur.
vi. It may be a part of multiple neurofibromatosis.
vii. Cyto-albuminous dissociation.
viii. X-ray skull (Towne's view) may show widening, tunneling or erosion
of the acoustic meatus.
167
INVESTIGATON OF A CASE OF BRAIN TUMOUR:
1. Good History.
2. Neurological & General Examination.
3. Fundus examination.
4. Visual field mapping.
5. Examination of the Head,
6. Plain x-ray skull:
* General signs of increased ICT.
* Lateralizing signs.
* Localizing signs.
7. CT/MRI Brain.
8. Magnetic Resonance Angiography (MRA):
* General signs of increased ICT.
* Lateralizing signs.
* Localizing signs.
9. Conventional Angiography
10. EEG.
11. Radiography of the lungs.
PRIMARY INTRACRANIAL TUMORS
Tumor
Clinical Features
Glioblastoma
multiforme
Presents commonly with nonspecific
complaints & increased intracranial
pressure. As it grows, focal deficits
develop.
Astrocytoma
Presentation similar to glioblastoma
multiforme but course more protracted,
often over several years. Cerebellar
astrocytoma may have a more benign
course.
Medulloblastoma
Treatment & Prognosis
Course is rapidly progressive,
with poor prognosis. Total
surgical removal is usually not
possible. Radiation therapy &
chemotherapy may prolong
survival.
Prognosis is variable. By the
time of diagnosis, total excision
is usually impossible; tumor
often is not radiosensitive. In
cerebellar astrocytoma, total
surgical removal is often
possible.
Seen most frequently in children.
Treatment consists of surgery
Generally arises from roof of fourth
combined with radiation
ventricle & leads to increased intracranial therapy & chemotherapy.
pressure accompanied by brainstem &
cerebellar signs.
168
Ependymoma
Glioma arising from the ependyma of a Tumor is not radiosensitive &
ventricle, especially the fourth ventricle; is best treated surgically if
leads early to signs of increased
possible.
intracranial pressure. Arises also from
central canal of cord.
Oligodendroglioma
Slow-growing. Usually arises in cerebral Treatment is surgical & usually
hemisphere in adults. Calcification may successful.
be visible on skull x-ray.
Brainstem
glioma
Presents during childhood with cranial
Tumor is inoperable; treatment
nerve palsies & then with long tract signs is by irradiation & shunt for
in the limbs. Signs of increased
increased intracranial pressure.
intracranial pressure occur late.
Cerebellar
Hemangioblastoma
Presents with dysequilibrium, ataxia of Treatment is surgical.
trunk or limbs, & signs of increased
intracranial pressure. Sometimes
familial. May be associated with
retinal & spinal vascular lesions,
polycythemia, & renal cell carcinoma.
Pineal tumor Presents with increased intracranial
pressure, sometimes associated with
impaired upward gaze (Parinaud's syndrome) & other deficits indicative of
midbrain lesion.
Ventricular decompression
by shunting is followed by
surgical approach to tumor;
irradiation is indicated if
tumor is malignant. Prognosis
depends on histopathologic
findings & extent of tumor.
Originates from remnants of Rathke's
Craniopharyngioma pouch above the sella, depressing the
optic chiasm. May present at any age
but usually in childhood, with
endocrine dysfunction & bitemporal
field defects.
Treatment is surgical, but
total removal may not be
possible.
169
Acoustic
neurinoma
Ipsilateral hearing loss is most
common initial symptom. Subsequent
symptoms may include tinnitus,
headache, vertigo, facial weakness or
numbness, & long tract signs. (May be
familial & bilateral when related to
neurofibromatosis.) Most sensitive
screening tests are MRI & brainstem
auditory evoked potential.
Treatment is excision by
translabyrinthine surgery,
craniectomy, or a combined
approach. Outcome is usually
good.
Meningioma
Originates from the dura mater or
arachnid; compresses rather than
invades adjacent neural structures.
Increasingly common with advancing
age. Tumor size varies greatly.
Symptoms vary with tumor site e.g.,
unilateral exophthalmos (sphenoidal
ridge); anosmia & optic nerve
compression (olfactory groove).
Tumor is usually benign & readily
detected by CT scanning; may lead to
calcification & bone erosion visible on
plain
x-rayswith
of skull.
Associated
AIDS & other
immunodeficient states. Presentation
may be with focal deficits or with
disturbances of cognition &
consciousness. May be indistinguishable from cerebral toxoplasmosis.
Treatment is surgical. Tumor
may recur if removal is
incomplete.
Primary
Cerebral
lymphoma
Treatment is by whole brain
irradiation; chemotherapy
may have an adjunctive role.
Prognosis depends upon CD4
count at diagnosis.
CHILDHOOD BRAIN TUMORS
Childhood brain tumors include the following:
A. Posterior Fossa Tumors
 Medulloblastomas
 Brainstem gliomas
 Ependymomas
 Cerebellar astrocytomas
B. Supratentorial Astrocytomas Grades I, II, III. & IV
C. Others:
 Craniopharyngiomas
 Optic gliomas
170
Posterior Fossa Tumors
Medulloblastoma
Cerebellar
Astrocytoma
Ependymoma
Brainstem Glioma
Location
Midline of
cerebellum
Midline or cerebellar 4th ventricle
hemisphere
Pons, medulla,
mesencephalon
Age
< 2-15
5-15
< 2.4
2-9
Signs &
Symptoms
Ataxia, headache,
vomiting, papilledema
Ataxia. headache.
vomiting, papilledema
Ataxia headache,
vomiting,
papilledema
Ataxia, cranial
nerve palsies, long
tract signs, papilledema late or
absent
Pathology
Homogeneous
round cells
Microcystic or cystic Variable lowVariable gliowith nodule
grade or malignant blastoma multiform
or pilocystic
astrocytoma
Treatment
Surgery
Craniospinal
Radiation +
Chemotherapy
Surgery. Local
radiation if not
totally removed
Surgery
Radiation
? Chemotherapy
± Biopsy
Local radiation
? Chemotherapy
5-year
Survival
40-50%
>90%
30-40%
<20%
Treatment Approaches Include:
1) Surgery e.g. in cerebellar astrocytomas, craniopharyngiomas, &
meningiomas.
2) Radiotherapy
3) Chemotherapy
4) Surgery + Radiotherapy
5) Surgery + Radiotherapy + Chemotherapy
SPINAL CORD COMPRESSION



Metastases in the spine with erosion into the epidural space & spinal
cord compression, frequently come from carcinomas of the lung, breast,
& prostate.
Direct extension by paraspinal tumor occurs with lymphomas & some
retroperitoneal tumors.
Intramedullary & intradural extramedullary metastases are rare.
171
Clinically:
 Back pain, local or radicular, is the initial symptom in 95% of cases.
 Weakness, sensory loss, & autonomic dysfunction appear weeks or
months later.
Diagnosis is based on:
 History & neurologic examination:
 Back pain, spinal percussion tenderness, sensory levels, motor deficits.
 Plain X-rays of the entire spine
 Myelography for precise localization of epidural tumor.
 CSF examination for malignant cells, as carcinomatous meningitis may
co-exist.
 Body CT scanning at the level of cord compression is useful prior to
radiotherapy in selected cases. MRI of the spine is more informative if
available.
Treatment:
(1) Radiotherapy
(2) Decompressive laminectomy in selected cases, or removal of the affected
vertebral body & epidural tumor in highly selected patients who fail to
respond to radiotherapy.
(3) Dexamethasone Therapy:
/ Decadron Vial (100 mg IV bolus)
/ Millicorten 1 mg Tab.
Or: Dexazone 0.5 mg Tab.
(24 mg orally every 6 hrs for 2 days, then rapidly taper)
N.B.:
Atlantoaxial subluxation is a serious complication:
 Lateral X-rays of cervical spine are diagnostic.
 Early lesions are treated by a soft cervical collar + radiotherapy.
 Completed subluxation requires reduction by cervical traction +
posterior cervical fusion + radiotherapy.
 If surgery is not possible, try support of the head & neck in a collar +
steroids + radiotherapy.
Spinal Subdural Hematoma may occur in thrombocytopenic cancer pts.
 Platelet transfusion & high doses of dexamethasone are needed
172
CARCINOMATOUS MENINGITIS

It is a frequent complication of melanoma & cancer breast, acute
leukemias & lymphomas.
 Clinically: headache, mental & behavioral changes, low back pain &
radicular pain in the extremities followed weeks later by stupor, multiple
asymmetric cranial nerve palsies, & flaccid areflexic quadri-paresis,
with sphincteric disturbance.
 CSF examination may reveal malignant cells, elevated pressure,
increased protein, lymphocytic pleocytosis, ± decreased glucose.
 Myelography may reveal tumor nodules in the subarachnoid space.
 CT scanning may show contrast enhancement along the basal cisterns.
Treatment:
(1) Irradiating the area of maximal neurological dysfunction.
(2) Lumbar intrathecal & intraventricular chemotherapy using one of the
following drugs:
a. Methotrexate.
b. Cytosine arabinoside (Aracytin)
c. Thiotepa
PSEUDOTUMOR CEREBRI
IDIOPATHIC INTRACRANIAL HYPERTENSION
Modified Dandy Criteria for Idiopathic Intracranial Hypertension
 Signs & symptoms of  ICP (Papilledema or headache)
 No localizing findings on neurologic examination (except abducents
nerve palsy)
 Normal neuroimaging with no evidence of venous obstructive disease
 Increased intracranial pressure as measured by lumbar puncture
 Normal cerebrospinal fluid constituents
 Awake & alert patient
 No other cause of increased intracranial pressure
Symptomatic causes of intracranial hypertension
Space-occupying intracranial lesions
Venous hypertension
 Dural sinus thrombosis
 Arteriovenous malformations & dural fistulas
 Congestive heart failure/congenital heart disease
 Pulmonary hypoventilation
173
 Jugular vein or vena cava obstruction
 Idiopathic thrombocytopenic purpura
 Iron-deficiency anemia
 Gammopathies
Chronic uremia
Endocrine disorders
 Treated hypothyroidism in children
 Alimentation of deprivational dwarfism
 Hypoparathyroidism
 Addison’s disease
Hydrocephalus
Toxins
 Chlordecone
 Hypervitaminosis A
 Nalidixic acid
 Danazol
 Lithium
 Amiodarone
 Tetracycline or minocycline
 Nitrofurantoin
Infectious disease
 Meningitis: viral (coxsackievirus B), bacterial, fungal
 Brucellosis
 Lyme disease
 Guillain-Barré syndrome
 Parasites: malaria, sandfly fever, trypanosomiasis
 Torulosis
 Neurocystercercosis
 Syphilis
Sarcoid
Treatment of Pseudotumor Cerebri
Diet & Lifestyle:
 Encourage pts to lose wt
 Avoid excessive water intake
 Salt restriction
 A tyramine-free diet ↓ headache
 A rice diet may be effective
174

Pts should be seen monthly for testing visual acuity, visual fields &
ophthalmoscopy
Drug Treatment
Diuretics:
Carbonic anhydrase inhibitors:
/ Acetazolamide (Diamox, Cidamex),
(250-500 mg /d, ↑ as tolerated to 1000-4000 mg in divided doses bid-tid)
Or: Furosemide (Lasix)( Adults: 20-80 mg /d; Children: 1 mg / kg /d)
Or: Chlorthalidone (Hygroton 50 mg tab.)
(12.5-50 mg /d, or 2 mg /kg up to 120 mg /d, or 200 mg / other day)
Corticosteroids:
/ Prednisone (Hostacortin)
(Oral 2 mg / kg /d. for 2 wks, with tapering over another 2 wks)
Or: Methylprednisolone (Solu-Medrol)
(IV 250mg qid for 5days, with an oral taper beginning at 80mg over 48wks)
Interventional Procedures
Lumbar puncture (L.P.) & Drainage.
 Pt is placed in a lateral decubitus position
 L.P. (in 4th or 5th lumbar interspace)
 Measure the CSF pressure by a manometer
 Remove CSF for cell, protein, glucose, & culture studies
 Remove large volumes to ↓ closing pressure to < 200 mm H 2O
Surgery (to preserve vision)
 Lumbar peritoneal shunting
 Optic nerve sheath decompression
 Bariatric surgery (to reduce wt.):
 Horizontal gastroplasty, or
 Vertical-banded gastroplasty
Emerging Therapies:
 Octreotide (Sandostatin):
 A long-acting somatostatin analogue (a potent inhibitor of GH)
 Hyperbaric Oxygen
 Sch 23390:
 A serotonin receptor agonist (↓ CSF production in rats)
175
PSYCHOTROPIC DRUGS
(A) Anxiolytics (Minor Tranquilizers)
Apolorazepam tab
Atarax 10 mg & 25 mg tab
Ativan 1 mg & 2 mg tab
Buspar 5 mg, 10 mg tab
Calium tab
Calmepam 1.5 mg, 3 mg tab
EN 0.5 mg tab
Lexotanil 1.5 mg & 3 mg tab
Neo Opt tab
Neuril 2 mg & 5 mg tab,
Stesolid 10 mg amp., 5 mg supp.
Tranxene 5 mg & 10 mg cap.
Valinil 2 mg & 5 mg tab
Valpam 10 mg amp.
Valium, syrup
Xanax 0.25 mcg & 0.5 mcg tab
1-3 tabs or caps, daily or 1 tsp o.d.-tid
Neuril, 10 mg. amp
Stesolid 10 mg amp
Valpam 10 mg amp.
Valium 10 mg amp
Ad. & Older child: 2-20 mg by slow I.V. or I.M., Child > 5 yrs: status epilepticus: 1
mg / 2-5 min. up to 10 mg., muscle spasm & tetanus. 5-10 mg/3-4 hrs. Inf. > 30 days 5 yrs.: satatus: 0.2-0.5 mg slowly I.V. every 5 min. up to 5 mg., Muscle spasm &
tetanus: 1-2 mg slowly I.V. every 3-4 hrs.
(B) Neuroleptics (Major Tranquilizers)
Clopixol Acuphase amp. (1 amp i.m. prn)
Clopixol Depot amp.
Dobren 50 mg cap.
Dogmatil 50 mg cap. & 200 mg tab
Fluanxol 1 mg & 3 mg tab
Fluanxol Depot amp.
Haldol Amp.
Haloperidol tab
Imap amp.
Melleril Ret 30 mg & 200 mg tab
Modecate 25 mg amp.
Neurazine tab, amp, supp, dr.
176
Orap Forte 4 mg tab
Piportil 4 & 8 mg. amp.
Priadel 400 mg tab. (2-4 tabs 1 day)
Prianil 400 mg tab. (2-4 tabs 1 day)
Promacid 25 & 100 mg tab
Risperdal 1, 2, 3 & 4 mg tab
Safinace 1.5 mg & 5 mg tab.,
Sparine 25 mg/1 ml vial
Stelazine 1 mg, 2 mg & 5 mg tab
Tiapridal tab
Trilafon 4 mg & 8 mg tab
Zyprexa 5mg, 15 mg tab
1 tab or 1 cap or 1 sup o.d.-tid.
1 amp. i.m./ 1-4 wks
(C) Antidepressants
Anafranil 25 mg & SR 75 mg tab, amp.
Cipram 20 mg tab.
Gamonil tab
Ludiomil 10 mg, 25 mg & 75 mg tab.
Lustral 50 mg, 100 mg tab.
Motival tab
Parnate tab
Prothiaden 25 mg cap & 75 mg tab
Prozac 20 mg cap.
Survector 100 mg tab
Tofranil 10 mg, 25 mg tab
Trittico 50 mg & 100 mg tab.
Tryptizol 10 mg, 25 mg tab, vial
Vivalan 50 mg tab.
1-3 tabs or caps/d
(D) Hallucinogenics (Psychodysleptics)
Delysid Amp.: Lysergide (L.S.D.) 0.1 mg
(No accepted therapeutic indications for LSD. However, some claim that it is useful in
alcoholism, facilitation of psychotherapy & diminishing the need for opiates in
chronic cancer patients. Dosage: 1-2 ug/kg parenterally)
Sedatives & Hypnotics
I. Barbiturates
177
Luminal Amp.: Phenobarbitone Sodium 200 mg / ml
Sominal Amp.: Phenobarbitone Sodium 200 mg / ml
Gardenal Adult Amp.: Phenobarbitone Sodium 200 mg /ml
Gardenal Inf. Amp.: Phenobarbitone Sodium 40 mg /ml
Sominaletta Amp.: Phenobarbitone Sodium 40 mg / ml
(40-200 mg i.m. PRN)
Luminal 100 mg Tab.: Phenobarbitone Sodium
Gardenal 100 mg Tab.: Phenobarbitone Sodium
Gardenal 50 mg Tab.: Phenobarbitone Sodium
Gardenal 10 mg Tab.: Phenobarbitone Sodium
Sominal 60 mg Tab.: Phenobarbitone Sodium
Luminalette 15 mg Tab.: Phenobarbitone Sodium
Sominaletta 15 mg Tab.: Phenobarbitone Sodium
Phenobarbital 30 mg Tab.: Phenobarbitone Sodium
Adults: 50-100 mg o.d. – tid., Children: 10-15-30 mg o.d. - tid
Bellergal Retard Tab.: Belladonna Total Alkaloids 0.2 mg + Ergotamine Tartrate 0.6
mg + Phenobarbitone 40 mg (1 tab o.d. – bid)
Priscophen Tab.: Phenobarbitone 20 mg + Adiphenine HCl 10 mg + Tolazoline HCl
2.5 mg (1 tab tid)
Phenobarbitone Syrup: Phenobarbitone 66 mg / 100 ml (5 ml tid)
II. Benzodiazepines
Rohypnol Amp.: Flunitrazepam 2 mg / ml.
Tab.: 1-2 mg at bed time. Amp.: 1-2 mg slow i.m. or i.v
Rohypnol Tab.: Flunitrazepam 2 mg.
Noctamid 0.5 mg Tab.: Lormetazepam
Noctamid 1 mg Tab.: Lormetazepam
Loramet 0.5 mg Tab.: Lormetazepam
Loramet 1 mg Tab.: Lormetazepam
(0.5-1 mg at bedtime)
Mogadon Tab.: Nitrazepam 5 mg ( 5-10 mg at bedtime)
Levanxol 5 mg Cap.: Temazepam
Levanxol 10 mg Cap.: Temazepam
(5-10 mg at bedtime)
Normison 10 mg Cap.: Temazepam.
Normison 20 mg Cap.: Temazepam.
(10-20 mg at bedtime)
Rivotril Amp.: Clonazepam 1 mg
Rivotril Tab. 0.5 mg & 2 mg: Clonazepam
Rivotril Drops: Clonazepam 2.5 mg/ml (1 drop = 0.1 mg)
Tab.: Adults 2-4 mg at bedtime. Children: 0.5-l mg o.d. Amp: 1 mg slowly i.v. or i.m.
PRN
III. Non-Barbiturate Preparations
178
Calcibronat Amp.: Ca-Bromolactobionate 1.24 gm.
Calcibronat Eff. Tab.: Ca-Bromolactobionate 3 gm
Calcibronat Syrup: Ca-Bromolactobionate 1 gm / 5 ml.
Adults: 1 eff. tab or 15 ml syrup 1-3 times daily, or 5-10 ml slowly i.v. daily.
Children: 5-10 ml syrup bid or tid
Chloral Syrup: Chloral Hydrate 500 mg / 5 ml
Adults: 15-30 ml at bedtime. Children: 5-10 ml at bedtime
Sedocal Syrup: Ca-Bromide 5 gm + Orange-Flower Oil 10 gm + Peppermint Syrup 50
gm + Simple Syrup Add to 100 gm.
Aqua Baby Syrup: Sodium Citrate 50 mg + Potassium Bromide 30 mg + Tr.
Belladonna 0.01 ml + Tr. Cardamom Co. 0.05 ml + Spirit Mentha 0.75 ml /5ml.
Walirine Syrup: Na. Citrate 50 mg + K. Bromide 30 mg + Na. Benzoate 10 mg + Tr.
Belladonna 0.01 ml + Tr. Cardamon Co. 0.05 ml. + Glycerin 0.5 mg + Syrup
Orange 0.5 ml + Chloroform water 0.75 ml / 5 ml.
ScopyI Dry Syrup: Na. Bromide 50 mg + Scopolamine Methylnitrate 0.5 mg + NaCitrate 100 mg / 5 ml.
Calm Tea: Anise Fruit 0.6 gm + Fennel Fruit 0.6 gm + Caraway Fruit 0.6 gm +
Coriander Fruit 0.3 gm + Chamomile Flower 0.3 gm + Mentha Piperita Leaves
0.6 gm
(5-10 ml tid)
Skeletal Muscle Relaxants
Alloferin 10 mg. amp. (0.2 mg/kg i.v. initially, then give 1/5 of the dose according to
the need to maintain N.M. block). dose according to the need to maintain N.M. block)
Baclofen (Lioresal)10mg & 25mg tab (10-25 mg bid- tid)
Cholsuxinyl vial
Coltramyi tab, amp.
(1-2 amp. i.m.; 2-4 tab/d.)
Curarine amp. Flaxedil 40 mg amp.
(1.5 mg/kg i.v., initially, then give 1/5 of
the dose according to the need to
maintain N.M. block)
Idarelax tab.
(Ad.: 1-2 tabs. tid. with meals; child.: 1015 yrs: 1 tab. tid., 5-10 yrs: 0.5 tab. tid)
Myolastan tab.
(1 tab at bed time)
Myolgin cap.
(1-2 cap. tid after meals)
Norcuron amp.
Norflex tab, amp.
(1 tab tid or 1 amp. prn)
Norgesic tab.
(1-2 tabs tid.)
Paradrine tab
(1 tab tid)
Pavulon 2mg/ml amp.
(0.08 mg/kg i.v. initially, then give 1/5 of
the dose according to the need to
maintain N.M. block)
Relax cap.
(1 cap. Tid)
179
Sirdalud 4 mg tab
Somadril comp tab
Succinyl Choline amp.
Tracrium amp.
(1/2 - 1 tab tid.)
(1 tab tid)
(By I.V. infusion at 0.005-0.01
mg/kg/min. "0.3-0.6 mg/kg/hr" to
maintain N.M. block during surgery)
Narcotic Analgesics &
Narcotic Antagonistics
(A) Narcotic Analgesics
Acupan amp., tab.
Alphacamphine amp.
Fentanyl amp.
Morphine amp.
MST. continus tab
1 amp. i.m. or 1-2 tab. prn
1 amp. i.m. prn
1 amp. i.m. prn
1 amp. i.m. prn
Ad.: 1 tab/12 hrs., Child.: 0.2-0.8
mg/kg/12 hrs.
1 amp. i.m. prn
1 amp. i.m. prn
1 amp. i.m. or 1-2 cap. or 1 supp. prn
Ad.: 1 mg i.v. / 3-4 hrs, or 2 mg i.m. / 3-4
hrs
Nubain amp
Pethidine amp.
Propoxene amp, cap, supp
Stadol vial
(B) Narcotic Antagonistics
Anexate 0.5 mg/ 5 ml & 1 mg/10 ml amp.
Nalorphine amp.
Narcan amp.
Trexan 50 mg tab.
0.2 mg I.V. over 15 sec, then 0.1 mg i.v.
after 60 sec., my be repeated/60 sec. up
to a total of 1 mg
1 amp. s.c. prn
0.01 mg/kg I.V., repeated every 2-3 min
until respiration returns to normal & the
pt. responde to stimuli
1/2 tab. initially, then 50 mg daily or 100
mg/other day or 150 mg/3 days "Dont
attempt ttt until pt has remained 7-10
days opioid-free"
Anti-Gout Drugs
Allopurinol tab.
Apurol tab
Apurol Retard tab
No-Uric 100 & 300 mg tab
Colchicine tab.
Colmediten tab.
Lessuric 100 & 300 mg tab
Vrisal tab
180
Propen tab
Purinol tab
Zylopurinol tab
Zyloric 100 & 300 mg tab
1 tab bid-tid.
181