Download NEUROLOGIC INVESTIGATIONS

NEUROLOGIC INVESTIGATIONS
CSF Analysis
CSF (general info):
 Produced by choroids plexus of ventricles, absorbed through villi of arachnoid granulations that project into the dural
venous sinuses
 Production rate=.5 mL/min, total volume=150 mL, entire volume replaced every 5 hrs
Lumbar puncture:
 CI: space occupying lesion causing mass effect, increased ICP (LP can cause cerebral or cerebellar herniation)
 CT: should be performed prior to LP (except in cases of suspected meningitis)
 Complication: low pressure HA is most common (tx: pt should lie flat, increase liquid intake and caffeine)
 Technique:
o Pt position: lateral recumbent, legs flexed up over abdomen, pillow b/w legs
o Level: L3-4 vertebral interspace (@ level of anterior superior iliac spine); spinal cord ends at L1-2
o Needle: inserted w/ bevel facing up, directed slightly rostrally to coincide w/ downward angulation of
spinous process
Interpretation of CSF findings:
 Normal CSF: clear, colorless fluid w/ glc content 2/3 that of blood, trace ptn, <5 cells present (lymphocytes), opening
pressure of 60-150 mm water
RBCs
WBCs
Elevated ptn
Low glc
Oligoclonal bands
Positive EBV PCR
No xanthocromia
Xanthocromia (yellow
discoloration of supernatant of
spun CSF sample)
Polymorphs
Lymphocytes
Traumatic tap
SAH, hemorrhagic encephalitis
Bacterial or early viral infection
Infection (viral, fungal, mycobacterial), demyelination (MS),
CNS lymphoma
Infection, demyelination, tumor, age
Bacterial or mycobacterial infection
Demyelination (MS), CNS infections (Lyme Disease),
Noninfectious inflammatory processes (SLE)
Highly suggestive of CNS lymphoma in pts w/ AIDS or other
immunosuppressed states
CT
Concept
 Characterizes degree of x-ray attenuation by tissue. Attenuation is the removal (by absorption or scatter) of x-ray
photons and is quantified on an arbitrary scale (Hounsfield units) that is represented in shades of gray. Attenuation is
dependent on atomic number and physical density of tissue.
 Axial or coronal plane
 Iodine is contrast agent used (if pt has allergy, do MRI instead); contrast enhancement indicates local disruption of
blood-brain barrier
Utility
 Initial investigation used in variety of neuro d/o (HA, trauma, seizures, SAH, stroke)
 Investigation of choice for demonstrating fresh blood
Safety, tolerability, complications
 CI: pregnancy
MRI
Concept
 Uses radio frequency pulses (not x-rays). Images result from varying intensity of radio wave signals emanating from
tissue in which hydrogen ions have been excited by a radio frequency pulse
 Pt is placed in a magnet and then a radio frequency (RF) is administered
o TE=time to echo, the time interval at which the signal intensity is measured
o TR=time to repetition, the time between RF pulses
 Gadolinium is contrast agent used
Types
 T1-weighted
o Short TE/TR
o Fat is bright, water (CSF) is dark




o Used to obtain gadolinium-enhanced images
T2-weighted
o Longer TE/TR
o Water (CSF) is bright
FLAIR (fluid attenuated inversion recovery)
o Strong T2-weighted image, but one in which the signal from CSF has been inverted and is thus of low rather
than high intensity
o Used for demonstrating early or subtle T2 signal changes such as accumulation of edema
Susceptibility-weighted imaging
o Sensitive to the distruptive effect of a substance on the local magnetic field (calcium, bone, blood b/d
products ferritin and hemosiderin); areas of increased susceptibility appear black
Diffusion-weighted imaging (DWI)
o Demonstrates cellular toxicity w/ high sensitivity
o Most commonly used in diagnosis of acute stroke
o Areas or restricted diffusion appear bright on DWI
Utility
 Provides better anatomic definition
 Imaging of posterior fossa and craniocervical junction
 DWI is most sensitive for demonstrating early tissue ischemia and is useful in evaluation of pts w/ suspected stroke
Safety, tolerability, complications
 Modality of choice in pregnancy
 When contrasted imaging is required, MRI may be preferable to CT when there is a history of allergy to IV contrast
(gadolinium and iodine are not cross-reactive), or liver disease (gadolinium is not nephrotoxic)
 CI: metal objects, pacemaker/defibrillator devices, claustrophobia
EEG
Frequency patterns
 Alpha (8-13 Hz): posterior head regions in relaxed awake state w/ eyes closed
 Beta (14-30 Hz): frontal regions in relaxed awake state w/ eyes closed
 Theta (4-7 Hz): drowsiness/sleep
 Delta (.5-3 Hz): drowsiness/sleep
Technique
 Montage=pattern w/ which electrodes are connected to each other
o Bipolar: all electrodes are active, records difference in electrical activity b/w 2 adjacent electrodes
o Referential: electrical activity recorded beneath the active electrode relative to a distant or common average
electrode
Clinical utility
 Limitations
o Abnormal EEGs rarely are etiology-specific
o EEG records electrical activity of cortical neurons, and thus surface EEG may be insensitive to dysfunction
of deep structures
o Interictal EEG may only be abnormal in 30% of adults w/ epilepsy
 Abnormal patterns
o Focal arrhythmic or polymorphic slow activity in theta/delta range local pathology in underlying brain
o Generalized arrhythmic slow activity diffuse encephalopathy
o Sharp and spike wave discharges w/ or w/o accompanying slow wave interictal epileptiform findings
o Rhythmic spike or sharp and slow wave discharges or rhythmic slow waves focal or generalized
electrographic seizures
Nerve Conduction Studies (NCS)
 Electrical stimulus applied over a nerve, and recordings are made from surface skin electrodes
o Motor study: recording electrodes placed over end plate of a muscle innervated by nerve being stimulated;
nerve stimulated in at least 2 locations (distal, proximal) and distance b/w 2 sites is measured
 Distal latency=time interval b/w stimulation over distal portion of nerve and initiation of CMAP
 CMAP=compound muscle action potential
 Conduction velocity=calculated by measuring the difference in latency to CMAP initiation b/w
proximal and distal sites of stimualtion
o Sensory study: nerve stimulated at one site; SNAP (sensory nerve AP) recorded either at more proximal site
(orthograde study) or more distal site (antergrade study)
 Utility: assists localization of pathology w/in PNS
Distal latency
Conduction velocity
CMAP amplitude
EMG


Nerve Conduction Studies in Demyelinating and Axonal Neuropathies
Demyelinating
Axonal
Prolonged
Normal
Markedly reduced
Normal; maybe slightly reduced
Normal or mildly reduced
Reduced
Needle is inserted into individual muscles; recordings are made of muscle electrical activity upon insertion (insertional
activity), while muscle is at rest (spontaneous activity) and during contraction (volitional motor unit potentials)
Utility: assists localization of pathology w/in PNS
Insertional activity
Spontaneous activity
Volitional motor unit potentials
Recruitment
EMG in Neurogenic and Myopathic Disorders
Neurogenic
Myopathic
Increased (active denervation)
Usually normal
Increased (necrotizing myopathies)
Increased (active denervation)
Usually normal
Increased (necrotizing myopathies)
Large amplitude, polyphasic
Small amplitude, polyphasic
Reduced
Usually normal
THE APPROACH TO COMA AND ALTERED CONSCIOUSNESS
General
 Coma=state of unarousable unresponsiveness
 Describe alterations of consciousness in terms of a pt’s responses to various degrees of stimulation
 Glasgow Coma Scale—has prognostic value in head trauma pts, is reproducible, and easy to use
Clinical Approach to Altered Consciousness
1. ABCs: airway, breathing, circulation
2. Look for obvious clues to etiology
a. Medical problems (diabetes, hepatic failure, seizure disorder)
b. Circumstances in which patient was found
c. Check for meningeal signs
3. Try reversing common reversible etiologies
a. Naloxone (opiate OD)
b. Thiamine (EtOH)—give before dextrose, which can precipitate Wernicke’s encephalopathy
c. Dextrose
4. Check brainstem reflexes and look for focal signs
a. Focal signs present suspect structural cause neuroimaging
b. Focal signs absent suspect diffuse cause metabolic, toxic and/or infectious workup, neuroimaging
Examination
1. Mental status testing
a. Assess level of consciousness—an increasing gradient of stimulation should be applied and patient’s
responses recorded
b. For many pt’s, further cognitive testing may be impossible
2. Cranial nerve testing to assess brainstem function. Test these bs reflexes:
a. Pupillary—II (afferent) III (efferent)
b. Oculocephalic (doll’s eyes)—VIII (afferent) III, IV, VI (efferent)—forcibly turn head horizontally and
vertically and observe for conjugate eye movement in opposite direction (CI if cervical spine injury not r/o)
c. Caloric testing (if necessary, i.e., if turning head is CI or does not result in eye movement; never assume eyes
are immobile unless caloric testing has been done)—inject 50 mL ice water into each ear and observe for
conjugate eye deviation toward the ear injected
d. Corneal—V (afferent) VII (efferent)
e. Gag—IX (afferent) X/XI (efferent)
3. Motor tone
a. Motor tone
b. Decorticate posturing—bs dysfunction slightly more superior that that of decerebrate posturing
c. Decerebrate posturing—bs dysfunction
4.
5.
Muscle stretch reflexes, Babinski sign
Sensory testing usually limited to testing of light touch or pain sensation; applying nailbed pressure to each limb
may be useful in looking for gross sensory abnormalities
Differential Diagnosis
General:
 2 ways in theory in which consciousness can be depressed: dysfunctional bs (e.g. pontine hemorrhage) or bilateral
cerebral hemisphere dysfunction (e.g. hypoglycemia); BUT, unilateral cerebral hemisphere lesions, if large or severe
enough to cause swelling and compression of opposite hemisphere or downward pressure on bs, can also lead to coma
 General approach
o +/- bs reflexes indicates how deep coma is
o +/- focal signs narrows differential and guides workup
 Structural causes of depressed consciousness: acute ischemic stroke, acute intracranial hemorrhage,
brain tumor (w/ edema or hemorrhage), brain abscess
 Diffuse causes of depressed consciousness: metabolic, toxic, infectious, hypoxic-ischemic
(respiratory failure, cardiac arrest)
Laboratory and Radiographic Studies
 If STRUCTURAL cause is suspectedurgent head imaging, usually w/ noncontrast head CT; use CT rather than MRI
even if focal brainstem signs are found (d/t possibility of a large cerebral hemisphere lesion compressing the bs)
 If DIFFUSE cause suspected extensive metabolic, toxic, or infectious workup
o Blood testing: CBC, electrolytes, glc, liver function tests
o If infection suspected: chest x-ray, urinalysis, blood or urine culture, LP (BUT perform CT 1 st! to avoid
precipitating brain herniation)
o Head imaging usually needed b/c it may demonstrate signs of global hypoxic-ischemic injury, diffuse
cerebral edema, or bilateral lesions mimicking a diffuse process
 EEG nonspecific, but can be of use in helping to assess how deep a coma is, based on degree of background
slowing
Treatment and Prognosis
Treatment
 Diffuse metabolic, infectious, toxic medical management
 Structural neurosurgical intervention
 If ICP increased raise head of bed, hyperventilation, osmotic diuretics (mannitol), corticosteroids for edema a/w
brain tumors
Prognosis
 Depends on
o Etiology
o Age
Special Topics
1. Persistent vegetative state—may follow prolonged coma and is characterized by preserved sleep-wake cycles and
maintenance of autonomic functions w/ absence of awareness and cognition
2. Locked-in syndrome—awareness and cognitive function are preserved but almost complete paralysis occurs; cause by
large lesions in base of pons
3. Brain death—irreversible cessation of all functions of the entire brain, including the brainstem
a. Comatose
b. Absent bs reflexes
c. No spontaneous respirations even when PCO2 has been allowed to rise (apnea test)
NEURO-OPHTHALMOLOGY
Anatomy
 Coherent visual image: retinal axons lateral geniculate nucleus optic radiations in cerebral cortex primary
visual cortex (visual area 1, Brodmann’s area 17, striate cortex). V1 receives visual info from contralateral visual
hemifield.
 Perception of motion, depth, color, location, form: V1
o Associative visual cortex (areas 18, 29)
o Higher-order centers in posterior parietal and inferior temporal cortices
Visual Loss
+/- visual phenomena
1. Positive: brightness, shimmering, sparkling, hallucinations, shining, flickering, colors (suggest migraines or seizures)
2. Negative: blackness, grayness, dim vision, shade-obscuring vision (suggests stroke, TIA)
Clinical evaluation of visual loss
1. Visual acuity problem in refractive apparatus of eye or optic nerve
a. Hand-held Snellen
b. Pinhole (if VA is poor) if pinhole test improves VA, then problem is in refraction
c. If pt cannot read the letters count fingers perception of movement perception of bright light
2. Assessment of color vision
a. Ishihara plates (red desaturation seen early in optic nerve problems [esp. optic neuritis])
3. Test for afferent pupillary defects
a. Size of pupil (anisocoria?)
b. Light reaction, direct and consensual
c. Accomodation (near reaction)
4. Visual fields
5. Ophthalmoscopic exam retinal nerve fiber layer damage, optic atrophy, swollen disc, abnormal disc, vascular
lesions, retinal emboli
Monocular visual loss v. binocular visual loss
 Monocular suggests problems in eye, optic nerve, or chiasm
 Binocular suggest chiasm or retrochiasmal lesion
Visual Loss According to Localization
Lesion Level
Visual Field Defect
Optic nerve
Ipsilateral blindness
Chiasm
Bitemporal visual field defect (tunnel vision)
Optic tract
Contralateral homonymous hemianopia
Optic radiations (parietal)
Contralateral inferior homonymous quadrantonopia
Optic radiations (temporal, or Meyer’s loop)
Contralateral superior homonymous quadrantonopia
Optic radiations (both)
Contralateral homonymous hemianopia
Occipital cortex
Contralateral homonymous hemianopia w/ macular sparing
Disorders of the Pupil
Anatomy
 Light retinal ganglion cells optic nerve optic chiasm optic tract pretectal midbrain nuclei (EdingerWestphal) in rostral portion of 3rd nerve
 Efferent PNS fibers from EW travel w/ CNIII (thru cavernous sinus), ultimately synapsing in ciliar ganglion
sphincter pupillae (constrictor)
 SNS innervates dilator pupillae
o 1st order neuron: ipsilateral posterolateral hypothalamus down brainstem to IML @ C8-T1 spinal level
o 2nd order neuron: synapse in superior cervical ganglion
o 3rd order neuron: travel along internal carotid into cavernous sinus orbit
Clinical Assessment
Condition
Physiologic
anisocoria
Horner’s
syndrome
Signs/Symptoms
Asymmetric pupils
Etiology
Up to 25% of normal people
Ipsilateral miosis, ptosis,
ipsilateral anhidrosis
Impaired SNS innervation of pupil
CNIII palsy
(complete)
Mydriasis, ptosis,
ophthalmoplegia
Compression of CNIII: initially
produces dilated pupil w/o
compromising eye movements (b/c
PNS fibers run in outer part of CNIII,
while motor fibers are more internal)
Vascular compromise CNIII
Test
Amount of anisocoria does not change
with different illumination
Cocaine eyedrops that fail to dilate the
abnormal pupil (neg)
Hydroxyamphetamine eyedrops
allows for pharmacologic localization
if cocaine test is neg pre- v. postganglionic Horner’s (the pupil w/
post-gang Horner’s fails to dilate)
Adie’s pupil
(tonic pupil)
Dilated, with segmental
contraction and light-near
dissociation; anisocoria,
photophobia, blurred near
vision
Argyll
Robertson
pupils
Small, poorly reactive to
light but have preserved
near response
(accommodation normal)
ischemia (diabetes): normal, reactive
pupil, but palsy of ocular muscles
innervated by CNIII
Interruption of PNS supply arising
from ciliary ganglion
LND: normally pupil constriction to
light greater than to near stimulus;
opposite is true in LND (present when
there is a defect in light response as in
optic neuropathy or d/t aberrant
regeneration as in tonic pupil)
Syphilis
Abnormal Optic Disc
See pages 38-39 (table)
Note: painful vision loss=optic neuritis; sudden painless vision loss=ischemia
Diplopia
Strabismus
Comitant strabismus
Noncomitant strabismus
Phoria
Tropia
Some Terms Used to Describe Eye Misalignment
Misalignment of eyes
Misalignment is constant in all directions of gaze, and each eye has full ROM
(ophthalmologic problem)
Degree of misalignment varies w/ direction of gaze (neurologic problem)
Misalignment of eyes when binocular vision is absent
Misalignment of eyes when both eyes are opened and binocular vision is possible
Anatomy of Eye Movements (SO4 LR6 AR3)
 CNIII: dysfunction produces droopy dilated down and out; most common cause of CNIII dysfunction in older adults
include microvascular occlusion and ischemia d/t HTN, diabetes mellitus and atherosclerosis; patterns of dysfunction
include
o CNIII nucleus: bilateral ptosis, weakness of contralateral superior rectus
o Subarachnoid space: meningismus, constitutional sxs, CN defects
o Tentorial edge compression: depressed level of consciousness, hemiparesis, hx of trauma or supratentorial
mass lesion
 CNIV: superior oblique intorts, depresses, adducts; dysfunction is worse on downgaze and pts c/o diplopia while
reading and descending stairs and compensate w/ a contralateral head tilt (diplopia improves w/ head tilt away from
side of lesion
 CNVI: lateral rectus;
o Lesions produce esotropia
o Destruction of abducens nucleus in brainstem ipsilateral conjugate gaze palsy b/c of damage of
interneurons connected to contralateral 3rd nerve through the MLF (medial longitudinal fasciculus)
Clinical Evaluation of Diplopia
 Ask:
o Monocular or binocular?
o If binocular, is it horizontal or vertical?
o Is it worse near or far?
o Is the problem localized to an extraocular muscle (paresis or fatigue), brainstem MLF (internuclear
opthalmoplegia), or to the orbit itself?
 Tests
o Cover test: detects a tropia; pt fixates on a small target, then cover one eye; watch other eye; if eye makes a
refixation movement, this means that this eye was not aligned on the target; if eye moves nasally, pt has an
exotropia, and if temporally, and esotropia; e.g. CNIII palsy exotropia and hypotropia of paretic eye;
CNVI palsy produces esotropia of affected eye
o Alternate cover test: detects phoria (eso or exophoria); phoria do not cause diplopia b/c eyes are aligned
when both are opened simultaneously
o Park’s 3-step test: detects CNIV palsy; 1) hypertropia of paretic eye 2) which increases when the pt looks to
the opposite side and when 3) pt tilts the head to the same sider
o Oculocephalic maneuver (doll’s eye test)
o
Saccades: rapid conjugate movement of the eyes between objects (fingertips); in general d/o of eye
movements will produce slowness of saccades in the direction of the paretic muscle
Pupillary size and reflexes
Periocular signs or proptosis
o
o
Horizontal Gaze
 Internuclear opthlamoplegia (INO)
o MLF connects CNVI nucleus w/ contralateral CNIII nucleus
o Clinical characteristics of a RINO
 Inability to adduct R eye in left lateral gaze
 Nystagmus of abducting left eye
 Adduction during convergence is maintained b/c this action does not depend on the MLF
o Bilateral INOs can be seen in Wernicke’s encephalopathy
 “One and a half syndrome”
o D/t lesion of ipsilateral MLF + ipsilateral PPRF (paramedian pontine reticular formation=premotor substrate
for ipislateral horizontal gaze) OR CNVI nucleus; causes are MS and brainstem ischemia
o Clinical characteristics
 Ipsilateral lateral gaze palsy and INO in contralateral lateral gaze
 The only movement present in the lateral plane is abduction of contralateral eye
Vertical Gaze
 Controlled by rostral interstitial nucleus of the MLF (riMLF) located in the pretectal area near CNIII nucleus. Fibers
controlling upgaze from riMLF cross to contralateral side using posterior commisure to communicate w/ inferior
oblique and superior rectus subnuclei of CNIII complex
 Abnormal vertical gaze movements d/t dorsal midbrain syndromes
o Parinaud’s syndrome: upgaze disturbance, convergence-retraction nystagmus on attempted upgaze, lightnear dissociation of pupils (d/t pineal tumor compressing dorsal midbrain)
o Skew deviation: vertical tropia d/t brainstem or cerebellar lesion; hypotropic (lower) eye often on side of
lesion
Supranuclear Eye Movements
Saccades: rapid eye movements that redirect the fovea to a new target
 Voluntary horizontal saccades originate in frontal eye field and superior colliculus contralateral to the direction of
gaze; these areas have direct connection to contralateral PPRF
 Vertical saccades may also originate in frontal eye fields or superior colliculus but connect to contralateral riMLF
 Ocular motor apraxia=inability to produce saccades
 Abnormal saccades: hypermetric (overshoot), hypometric (undershoot); see table 4-6 on p. 42
Pursuit Movements: permit eyes to conjugately track a moving visual target to keep it in focus
 Control is hemispheric and ipsilateral
Vestibulo-ocular Reflex (VOR): coordinates eye movements w/ head movement, allowing visual image to not slip during
head movement
 Semicircular canals (rotation) and otoliths (linear acceleration)vestibular nucleiabducens nuclei CN III/IV via
MLF
 Abnormalities of VOR result in nystagmus:
Nystagmus: rhythmic to-and-fro movement of the eyes
 Types
o Rhythmic
o Jerk: eye drifts away from fixation in a pursuit-like movement and returns with a fast, saccadic movement;
the direction of nystagmus is named by the direction of the saccadic fast component
 Etiologies
o Congenital
o Physiologic
o CNS dysfunction, peripheral vestibular loss, visual loss
 Peripheral: usually unidirectional, w/ fast phase away from the side of the lesion; combines
horizontal and torsional movements and is inhibited by fixation; tinnitus or deafness often present;
severe vertigo; duration is short by recurrent
 Central: normally bi-directional, often purely horizontal, vertical, or torsional, and not inhibited by
fixation; tinnitus and deafness rarely present; mild vertigo; duration may be chronic
 See table on p. 43 (4-7)
THE APPROACH TO WEAKNESS
Approach to Weakness Flowchart
1. Make sure that true weakness (i.e. decreased strength) is the complaint (NOT general sense of fatigue, NOT clumsy or
numb, NOT a limb that is too painful to move)
2. ID which muscles are weak
3. Determine the pattern of weakness (muscle, NMJ, nerve, root, plexus, cord, brain?)
4. Look for associated signs/symptoms (numbness, tingling, painful; reflexes?). Are they consistent with localization?
5. Consider the differential diagnosis of d/o w/in your localization
6. Use lab tests and EMG/NCS if needed
a. Blood tests or neuroimaging
b. EMG/NCS to further localize problem to a particular segment of the peripheral nervous system
Differential Diagnosis of Weakness
Anatomic
Location
Primary Muscle
NMJ
Peripheral Nerve
Mononeuropathies
Mononeuropathy
Multiplex
Polyneuropathy
Nerve Root
Pattern of Weakness
Associated Si/Sx
Lab Studies
Symmetric, proximal
weakness and can affect neck
muscles (not usually affected
in a brain or nerve lesion)
Distal muscles affected later
or not as severely
Proximal weakness; some
can lead to ptosis and
weakness of extraocular,
bulbar, and neck muscles
Fluctuation of weakness is
important—degree may
change from hr to hr, may be
worse after using muscles or
toward end of day and
improve after resting or in
the morning (fatiguability) or
improve after exercise
Muscle pain (if
process is
inflammatory, e.g.
polymyositis)
Sensory si/sx are not
present
Sensory si/sx are not
present
ANS features
sometimes present
Serum CK level is
elevated in some d/o
EMG may show
characteristic
“myopathic” pattern
Weakness of muscles
innervated by a single
peripheral nerve
Sensory sxs (e.g.
numbness, tingling,
pain) in distribution of
the peripheral nerve
Characteristically a/w
pain
EMG/NCS: confirm
clinical suspicion of
problem w/ peripheral
nerve
NCS: does the
pathologic process
involve axons or
myelin of the nerve
EMG: relative acuity
or chronicity of d/o
Certain systemic d/o can lead
to dysfxn of multiple
peripheral nerves in
succession
Peripheral nerves are all
affected diffusely, w/ dysfxn
occurring in longest nerves
1st (progression of weakness
from distal to proximal,
symmetric)
Usually have
associated sensory loss
and depressed or
absent reflexes,
especially in distal
extremities
EMG/NCS can
demonstrate nearly
pathognomonic
findings for some d/o
Some d/o have
specific serum
markers (anti-AChR
Igs in myasthenia
gravis)
Differential
Diagnosis
Acquired
(myopathies) d/t
inflammatory or toxic
etiology
Congenital (muscular
dystrophies)
Myasthenia Gravis
Lambert-Eaton
Myasthenic
Syndrome
Usually occur d/t
entrapment (e.g.
Carpal Tunnel
Syndrome)
A/w systemic
vasculitis, metabolic,
or rheumatologic d/o
Demyelinating
polyneuropathies can
be hereditary
(Charcot-MarieTooth) or acquired
(Guillain-Barre)
Radiculopathy
Dysfxn of single nerve root
Pain or tingling
radiating out from
back or neck
Objective sensory loss
rare in d/o of single
nerve root b/c of
overlap from adjacent
roots
If nerve root subserves
a particular muscle
stretch reflex, that
reflex may be
depressed or absent
EMG/NCS can
confirm that nerve
roots are the culprit;
can differentiate b/w
root v. peripheral
nerve problem
MRI (spine): for
single rad., to r/o
structural etiologies
LP: for polyrad. to
look for infectious or
inflammatory
processes
Herniated discs
Shingles
Inflammation
Infection
Polyradiculopathy
Dysfxn of multiple nerve
roots
Multiple muscles in a limb
are weak and do not conform
to a particular nerve root or
peripheral nerve pattern
Sensory findings (in
distribution of one or
more roots or nerves)
Dropped reflexes
EMG/NCS: confirms
the localization
MRI of brachial or
lumbosacral plexi to
r/o mass lesions
Spinal Cord
Weakness in UMN pattern
below lesion, and in a nerve
root pattern at lesion level
MRI (spine): r/o
structural etiologies or
demonstrate intrinsic
inflammation
LP: evaluate
infectious or
inflammatory etiology
Cerebral
Hemispheres/
Brainstem
Hemisphere:
contralateral side in UMN
pattern
Parasagittal: contralateral
leg
Lateral hemisphere: arm and
face
Deep hemisphere (e.g.
internal capsule): legs, arms,
and face
Base of pons: ipsilateral
face, contralateral arm, leg
(crossed signs) b/c
descending motor fibers to
face have crossed at that
level by those to body have
not
Maybe sensory loss
below level of lesion
d/t interruption of
ascending tracts
Reflexes below lesion
are typically increased,
and Babinski may be +
Bladder/bowel
incontinence maybe
Cognitive signs
Idiopathic
inflammation,
radiation, metastases,
hemorrhage, trauma
Diabetic pts
characteristic
lumbosacral
plexopathy (diabetic
amyotrophy)
Inflammation (e.g.
transverse myelitis)
Infarction
Compression
ALS (degeneration of
corticospinal tracts
and anterior horn
cells)
Plexus
Brachial
Lumbosacral
Left hemisphere:
aphasia, apraxia
Right hemisphere:
neglect, visuospatial
dysfunction
Brainstem: CN
dysfunction
CT or MRI
Stroke, demyelinating
disease, traumatic
injury, tumor,
infection
THE SENSORY SYSTEM
Anatomy of Sensory Pathways
Modality
Tract
Pain and
temperature
Spinothalamic
tract
Proprioception,
vibration, light
touch
Dorsal columns
1st order
neuron
Synapse at
level of
dorsal horn of
spinal cord
2nd order
neuron
Cross and
travel
contralaterally
in STT
Terminates in
thalamus
Legsarms
Mediallateral
Travel
ipsilaterally
in dorsal
column
system
reaching 2nd
order neuron
at level of
medulla in
nuclei gracilis
and cuneatus
Axons from
nuclei gracilis
and cuneatus
cross at lower
medulla to
form the
medial
lemniscus
Nucleus gracilis
Nucleus cuneatus
Medial lemniscus
Facial sensation
Somatotopic
Arrangement
Sacralcervical
Lateralmedial
CNV
Legsarms
Lateralmedial
3rd order
neuron
Cortical
projections to
postcentral
cortex
Fiber type
Thinly
myelinated
and
unmyelinated
slowly
conducting
fibers (Adelta and C)
Heavilymyelinated Aalpha and Abeta)
Terminates in
thalamus
Examining the Sensory System
 Touch wisp of cotton
 Pain pin
 Joint position sense/proprioception moving great toe up and down, moving object up or down on skin and asking pt
the direction of movement
 Vibration 128-Hz tuning fork applied to toes and other bony structures
 Temperature cold tuning fork
Sensory Abnormalities: Terms
Paresthesia
Dysesthesia
Hyperesthesia
Hypoesthesia
Allodynia
Dissociated sensory
loss
Negative symptoms
Positive symptoms
Abnormal sensation described by pt as tingling, prickling, pins and needles, etc.
Unpleasant sensations triggered by painless stimuli
Increased sensitivity to sensory stimuli
Diminished sensitivity to sensory stimuli
Pain provoked by normally innocuous stimuli
Loss of one of sensory systems w/ preservation of another one, e.g. in a central cord syndrome, you
will get loss of pain and temperature (STT) w/ preservation of light touch, proprioception, and
vibration sense (dorsal columns)
Numbness, loss of cold/warm sensation, blindness, deafness (d/t disruption of nerve excitation)
Pain, paresthesias, visual sparkles, tinnitus (d/t excitation or disinhibition)
Approach to Pt with Sensory Loss




Recognize sensory abnormality by modality
Judge level at which the abnormality is produced
Establish a diff dx of pathologic processes that can affect the particular modality/sensory system
Establish the cause: primary neurologic disease OR systemic disease
Patterns of Sensory Loss According to Localization
Site of Lesions
Sensory Findings
Peripheral nerve
Loss of LT, T, PP, proprioception in
influenced area
Root
Loss of all sensory modalities in a
dermatomal distribution
Plexus
Sensory loss in distribution of 2 or more
peripheral nerves
Spinal cord
Sensory level: bilateral loss of all
sensory modalities
Sensory dissociation
Contralateral hypesthesia and ipsilateral
loss of proprioception (Brown-Sequard
syndrome)
Proprioceptive loss and corticospinal
tract involvement
Saddle anesthesia
Ipsilateral facial numbness and
contralateral body numbness
Brainstem
Other Neurologic
Abnormalities
Associated weakness in
muscles innervated by that
nerve; distal muscle
weakness, atrophy, areflexia
(LMN signs)
Weakness in a myotomal
distribution, atrophy,
segmental hyporeflexia
Muscle weakness that cannot
be localized to a single nerve
or root
Paraplegia, tetraplegia;
initially areflexia, then
hyperreflexia below the
lesion and Babinski sign
(UMN signs)
Examples
Peroneal neuropathy
Median and ulnar
neuropathies
L5 radiculopathy
Cervical radiculopathy
Brachial plexopathy d/t
trauma, inflammation,
infiltration, etc.
Myelopathy
Central cord syndrome
Brown-Sequard syndrome
Alternating hemiplegia; CN
findings, INO, ataxia
Thalamus
Hemibody anesthesia
May have motor findings
Posterior limb of
internal capsule
Hemibody anesthesia
Hemiplegia
Cortex
All modalities affected on contralateral
side
Sensory neglect
Agraphesthesia
Psychogenic
Hyperesthesia for one modality in one
area w/ anesthesia for another modality
in same area; changing sensory findings
Nonphysiologic sensory level changes
(abrupt midline changes, vibration
asymmetry over forehead)
Any
Posterior circulation
strokes
Tumor
Lacunar stroke
Hemmorhage
Lacunar stroke
Hemorrhage
Tumor
Parietal stroke
Hemorrhage
AVM
Psychogenic (a diagnosis
of exclusion)
VERTIGO AND DIZZINESS
“Dizziness”
 Vertigo: illusion or hallucination of movement that is usually rotatory, but may be linear
 Light-headedness: feeling faint, refers to presyncopal state
 Dysequilibrium: sensation of imbalance or unsteadiness usually referable to legs rather than to a feeling inside head
Vertigo
 D/t acute asymmetry of neural activity b/w L and R vestibular nuclei
 Does NOT result from slow unilateral loss of vestibular fxn (acoustic neuroma) or from symmetric bilateral loss of
fxn (ototoxic drugs)
 Approach to determining etiology
o Periodicity/duration of sxs
o Positional or spontaneous sxs
o Peripheral or central origin
 Peripheral tinnitus, hearing loss; accompanying N/V more prominent
 Central diplopia, dysarthria, dysphagia, or other sxs of brainstem dysfunction; ability to walk or
maintain posture may be more impaired
Spontaneous Vertigo: Single prolonged episode
Vestibular neuronitis
Acute unilateral (complete or incomplete) peripheral vestibulopathy; sudden and spontaneous
onset of vertigo, N/V; onset over min-hrs, peak w/in 24 hrs, sxs improve gradually over several
days/wks; nystagmust is strictly unilateral and may be suppressed by visual fixation; recovery
represents central compensation for loss of peripheral vestibular fxn
Labyrinthine concussion
Can result from head injury; vertigo sometimes accompanied by hearing loss and tinnitus
Infarction of labyrinth, Blood supply to central/peripheral vestibular systems via vertebrobasilar system (posterior and
brainstem, or cerebellum
inferior cerebellar aa. and superior cerebellar artery) and to inner ear via anterior inferior
cerebellar artery (infarction of inner ear sudden onset of deafness or vertigo or both)
Brainstem or cerebellar stroke most important diff dx in pts w/ suspected acute vestibular
neuronitis; if a central-type nystagmus is present, then cerebellar or brainstem infarction w/
associated CN si/sx, weakness, ataxia, or sensory changes that clearly indicate a central process
Spontaneous vertigo: recurrent episodes
Meniere’s disease
Episodic vertigo w/ N/V; fluctuating, but progressive hearing loss, tinnitus, and a sensation of
fullness or pressure in ear; d/t an intermittent increase in endolymphatic volume
Perilymph fistula
D/t disruption of lining of endolymphatic system; pt reports hearing “pop” at time of a sudden
increase in middle ear pressure w/ sneezing, noseblowing, coughing, or straining, which is
followed by abrupt onset of vertigo
Migraine
Positional vertigo: peripheral
BPPV (benign positional Episodes precipitated by changes in position such as turning over in bed or looking upward;
paroxysmal vertigo)
attacks are brief (sec-min), and sxs typically begin after a few seconds’ latency following change
in position; d/t freely moving Ca++ carbonate crystals w/in one of semicircular canals; when
head is stationary, they settle in most dependent part of canal (posteriorly); w/ head movements,
they move slower than endolymph w/in which they lie, and their inertia once the head comes to
rest causes ongoing stimulation of the hair cells that results in the illusion of movement
(vertigo); Dix-Hallpike test is diagnostic (downbeating and torsional nystagmus); Epley
maneuver can be used to remove crystals from posterior canal
Syncope
 Transient LOC and postural tone d/t brain hypoperfusion
o Hypotension d/t cardiac dz
o Low intravascular volume
o Excessive vasodilation
 Presyncopal sxs include: light-headedness, HA, neck pain, blurring of vision, cognitive slowing, buckling of knees
 2 neurologic varieties (both involve ANS dysfunction)
o Neurogenic syncope (more common): acute hypotension results from transient failure of ANS cardiovascular
control; an acute hemodynamic rxn involving a reflex that is triggered by excessive afferent discharges from
arterial (including heart and great vessels) or visceral mechanoreceptors; efferent impulses via vagus lead to
cardio-inhibition and vasodepression that result in hypotension and bradycardia
 Micturition syncope trigger is rapid emptying of distended bladder
 Carotid sinus hypersensitivity trigger is compression of carotid sinus
 Neurocardiogenic syncope vigorous contraction of an underfilled ventricle
 Vasovagal syncope trigger is strong emotions or pain
o Neurogenic orthostatic hypotension: d/t persistent ANS failure; d/t intravascular volume depletion
(dehydration, Addison’s disease) or d/t inability to activate efferent SNS fibers appropriately upon
assumption of the upright posture; the underlying pathology is either primary or secondary ANS failure (e.g.
d/t diabetic ANS neuropathy), but the hallmark of both is failure to release noradrenaline upon standing;
consider contribution of drugs such as diuretics, antihypertensives, vasodilators, and antidepressants; drug
management to ameliorate sxs include midodrine and fludrocortisone
ATAXIA AND GAIT DISORDERS
Ataxia vs. Other Gait Disorders
 Ataxia: general term describing manifestations of dz of cerebellum or its connections
 Other: NOT ALL ataxia is cerebellar in origin, e.g. de-afferentation resulting from loss of position sense also results
in ataxia
 THUS: distinguish b/w cerebellar v. sensory ataxia
Ataxias
General
 Vermal lesions truncal/gait ataxia
 Hemispheric lesions ipsilateral limb ataxia
Si/Sx of Cerebellar Disease
 Dysmetria abnormality of range and force of movement; manifests as erratic, jerky movements w/ over- and
undershooting the target (limb or ocular dysmetria)
 Intention tremor rhythmic side-to-side oscillations of the limb as it approaches the target
 Dysdiadochokinesia abnormality of rate and rhythm of movement demonstrated by rapid alternating movement test
 Gait ataxia broad-based and unsteady, w/ inability to walk in straight line and tendency to lurch from side to side
 Truncal ataxia impaired control of truncal posture; when severe, unable to even site unsupported
 Dysarthria slow scanning and monotonous speech
 Nystagmus
Diseases
Disease
Cerebellar
hemmorhage/
infarction
Etiology
Hemorrhage/infarction
Characteristics
Abrupt onset of
vertigo, vomiting,
inability to walk
Postinfectious
cerebellitis
Typical b/w ages 2-7
following varicella or
viral infection
Episodic ataxias
Inherited mutations in
Ca++ and K+ channels
Underlying
gynecologic or small
cell lung cancer
Acute onset of limb
and gait ataxia,
dysarthria; severity
ranges from mild
unsteadiness to
inability to walk
Brief episodes of
ataxia, vertigo, N/V
Acute/subacute onset
of pancerebellar
syndrome w/ truncal,
gait, limb ataxia;
dysarthria; ocular
dysmetria and
nystagmus
Vermis bears brunt of
damageprogressive
gait and truncal ataxia
evolving over wks-mo
Paraneoplastic
cerebellar degeneration
Alcoholic cerebellar
degeneration
Long-standing alcohol
abuse; most common
cause of acquired
cerebellar degeneration
Friedrich’s ataxia
AR, inherited,
childhood onset
Progressive, affects
arms>legs; severe
dysarthria
AD spinocerebellar
degenerations
AD, inherited, onset in
young adult life
D/t trinucleotide CAG
expansion
Miller-Fisher
Syndrome
Variant of GuillainBarre syndrome
(mediated by postinfectious immune
process)
Insidious onset of
progressive
impairment of gat and
dysarthria in young
adult life
Triad of ataxia,
areflexia,
ophthalmoplegia
Ass. Si/Sx
Level of arousal may
be depressed if 4th
ventricle is
compressed w/
hydrocephalus or if
there is pressure on BS
Course + Prognosis
Medical emergency
Lasts a few weeks,
recovery is usually
complete
MRI usually normal
Anti-Yo, anti-Hu
autoantibodies in
serum
Disease peaks in a
period of weeks then
stabilizes, leaving
patient w/ profound
disability
Alcoholic
polyneuropathy
Cessation of drinking
and supplementation
of nutrition offer the
best (though limited)
chance of improving
Loss of reflexes,
spasticity, extensor
plantar responses,
impaired vibration and
position sense
Mild to moderate
cognitive decline is a
late feature
IgG anti-GQ1b
antibodies in serum of
>90%
Self-limiting
Usually full recovery
Gait Disorders
Gait Disorder
Hemiplegic
Anatomical
Location
Brainstem
Cerebral
hemisphere
Paraplegic
Spinal cord
Akinetic-rigid
Bihemispheral
Basal ganglia
Frontal
Frontal lobes
Subcortical
Waddling
Hip-girdle
weakness
Sensory
Ataxia
(Slapping)
Large-fiber
neuropathy
Dorsal
columns
Description
Pathology
Affected leg is stiff and does not flex at hip; leg is
circumducted, w/ tendency to scrape floor w/ toes;
arm is held in flexion and adduction and does not
swing freely; the spastic (paraparetic) gait is
essentially that of a bilateral hemiplegia; adductor
tone is increased, and legs tend to cross during
walking (scissoring gait)
Stroke, tumor, trauma
Demylination (MS),
transverse myelitis,
compressive myelopathy
Stooped posture; narrow-based, slow, shuffling gait w/
small steps and reduced arm swing; arms carried
flexed and slightly ahead of body; difficulty w/ gait
initiation; postural reflexes are impaired and pt may
take series of rapid small steps (festination) forward
(propulsion) or backward (retropulsion) in an effort to
preserve equlibrium
Flexed posture; feet may be slightly apart; gait
initiation is impaired, and word “magnetic” describes
difficulty lifting feet off ground; pt advances w/ small,
shuffling, and hesitant steps; w/ increasing severity, pt
may make abortive stepping movements in one place
w/o ability to move forward
D/t loss of proprioceptive input from feet; cautious,
wide-based gait; slow steps; contact w/ ground is
made by heel and forefoot then strikes floor w/ a
slapping sound (slapping gait); walking on uneven
surfaces or in dark is especially difficult
Diffuse anoxic injury
Parkinson’s Disease
Hydrocephalus, tumor,
stroke, neurodegenerative d/o
Binswanger’s disease
Muscular dystrophy, spi9nal
muscular atrophy, acquired
proximal myopathy
Vitamin B12 deficiency
Tabes dorsalis
URINARY AND SEXUAL DYSFUNCTION
URINARY
Anatomy/Physiology of Continence
Circuit
Circuit 1
Circuit 2 (spino-bulbarspinal)
Circuit 3


Connections
Dorsomedial frontal lobe to M region (pons)
Reflex arc starting in bladder projecting to M region
(pons) w/ outflow connections to PNS sacral spinal
motor nuclei
Spinal segmental reflex arc w/ afferents from detrusor
muscle to pudendal nucleus and efferent fibers to
striated sphincter muscles
Function
Volitional control of micturition
Pontine micturition center
o Areas
 M region stimulation decrease in urethral pressure, then rise in detrusor muscle pressure and
voiding; M region projects to sacral cord IML
 L region (same level) stimulation contraction of urethral sphincter (storage)
o Damage loss of inhibitory control over spinal reflexes (circuit 3) and as bladder distends, micturition reflex
is automatically activated w/o pt’s awareness or control, and detrusor hyperreflexia and incontinence occurs
Muscles of micturition
o Detrusor (smooth): PNS causes contraction; during filling it is inhibited, during micturition it is stimulated
o Internal urethral sphincter (smooth): SNS causes contraction; stim during filling, inh during micturition
o External urethral sphincter (skeletal): Somatic motor causes contraction; stim during filling, inh during mic
Evaluation of Incontinence
Urodynamic studies
 PVR (postvoid residual): normal<50mL; if increased implies poor bladder emptying (sphincter dyssynergia, atonic
bladder are common neurogenic causes)
 Cystometry: gives info re bladder compliance, capacity, volume at 1 st sensation and at urge to void; voiding pressure;
presence of uninhibited detrusor contractions
 Cystourethroscopy: assesses integrity of lower urinary system, and IDs important urethral and bladder lesions
 Retrograde urethrography
 Neurophysiologic studies: sphincter and pelvic floor EMG
Type of neurogenic
bladder
Spastic bladder
Capacity
Compliance
Other
Indicates…
Decreased
Reduced
Uninhibited detrusor
contractions
Atonic bladder
Increased
Increased
Low voiding pressure
and flow rate
UMN problem d/t
frontal lobe,
pontine, or
suprasacral spinal
cord lesion
LMN problem d/t
lesion at level of
conus medullaris,
cauda equina, or
sacral plexus, or
peripheral nerve
dysfunction
Type of
incontinence
Urge
Description
Etiology
Involuntary loss of
urine a/w strong
desire to void
(urgency)
Detrusor instability
(DI)
Detrusor hyperreflexia
(if DI d/t neurologic
problem)
Stress
Involuntary loss of
urine during
coughing, sneezing,
laughing, or
activities that
increase intraabdominal pressure
Urge + stress
Involuntary loss of
urine a/w
overdistension of
bladder; constant
dribbling
Weak pelvic floor
muscles
Mixed
Overflow
Underactive or
acontractile detrusor
(drugs, diabetic
neuropathy, lower
spinal cord injury, or
radical pelvic surgery
that interrups
innervation of detrusor)
Clinical
expression
Spastic
bladder
Patient
population
Strokes,
suprasacral
spinal cord
lesions, MS
Associated si/sx
Detrusor-sphincter dyssynergia
(DSD): inappropriate
contraction of external sphincter
w/ detrusor contraction
urinary retention, vesicourethral
reflux, and renal damage
Multiparous
women
Atonic
bladder
Incontinence in the Neurologic Patient
 Stroke and spinal cord disease usually produce an UMN bladder or spastic bladder w/ or w/o sphincter dyssynergia
o Supraspinal dz
 Stroke
 Parkinson’s (40-70%)
o Spinal cord dz
 Spinal cord injury
 Multiple sclerosis (75%)
 Small-fiber neuropathies can produce a neurogenic atonic bladder w/ high PVR (risk to upper urinary tract), e.g. as in
diabetic neuropathy
Treatment of Urinary Incontinence
Goals
 Preservation of upper urinary tract fxn
 Improvement of pt’s urinary symptoms that impair quality of life
Treatments
Disorder
Urge incontinence (spastic bladder)
Stress incontinence
Atonic bladder w/ overflow
incontinence
Detrusor dyssynergia
Treatments
1. Anticholinergic agents: Tolterodine (Detrol), Oxybutynin (Ditropan),
Propantheline
2. TCAs: imipramine
3. Desmopressin spray/tabs (DDAVP): used to treat diabetes insipidus, but
produces a significant reduction in voiding frequency
4. Intravesical capsaicin: for intractable detrusor hyperreflexia; has neurotoxic
effect on afferent C fibers that drive volume-determined reflex detrusor contractions
1. Alpha-adrenergic agonist drugs: Phenylpropanolamine, Pseudoephedrine (these
stimulate smooth muscle alpha-adrenergic receptors contraction)
2. Estrogen therapy: adjunctive in post-menopausal women
1. Crede’s maneuver or valsalva maneuver to empty bladder
2. Intermittent self-catheterization (mainstay of long-term tx)
3. Pharmacotherapy usually not effective; cholinergic agent bethanecol (stimulates
cholinergic receptors, increasing detrusor muscle tone
1. Intermittent catheterization
2. Suprapubic catheterizaition
3. Sacral nerve stimulation
ERECTILE DYSFUNCTION
 Persistent inability to attain or maintain penile erection sufficient for sexual intercourse
 10-20 million American men affected
 80% of cases are biologic or organic in origin
Anatomy and Physiology
 Pudendal nerve: carries motor and sensory fibers that innervate the penis and clitoris
 PNS:
o Sacral cord S2-S4, erection
o Local mediators: NO, cGMP primarily released by PNS activity contributing to sustained erection
 SNS: cells in spinal cord levels T11-T12 through hypogastric plexus, ejaculation
Management
 Requires recognizing etiology (neurogenic, vascular, traumatic, endocrine, psychogenic, drugs) and treating
underlying cause
 Pharmacotherapy:
o Viagra (25-100 mg/day, 30-60 min before intercourse): SE include facial flushing, HA
o Intraurethral suppositories
o Intracavernosal injections of alprostadil (Caverject)
HEADACHE AND FACIAL PAIN
Pathophysiology
 Caused by disturbance/irritation of pain-sensitive structures in head
o W/in cranium: blood vessels, meninges, CN V, IX, X (brain parenchyma itself + bone are pain-insensitive)
o Outside cranium: periosteum of skull, muscles, nerves, vessels, subcutaneous tissues, eyes, ears, sinuses,
mucus membranes
 Pain pathway
o Irritation of pain-sensitive structure nociceptive input relayed to brain via CN V or upper cervical roots
 CNV: dermatomal distribution on face, anterior and middle cranial fossas (thus, painful stimulation
of structures in ant/mid fossas referred to eye or front or side of head)
 Upper cervical roots: dermatomal on posterior surface of head and neck and ears, posterior fossa
(thus, painful stimulation of structures in posterior fossa referred to back of head and upper part of
neck)
3 General Classes of HAs
 Primary (migraine, cluster, tension) not a/w underlying structural cause
 HA a/w serious neurologic dz (brain tumor, meningitis, aneurysm)
 HA/pain from structures in skull and face (eyes, sinuses, mouth)
History of the Presenting HA
Hx Component
Onset and frequency of pain
Location of pain
+/- prodromal symptoms?
Precipitating factors
Description of pain
Associated sx/neurologic signs
Example
Unilateral cluster, migraine
Bilateral tension
Scintillating scotoma prior to classic migraine
Alcohol as a trigger for cluster
Nitrite-containing food (hot dog/salami), tyramine-containing cheese as migraine trigger
Lancinating/shooting pain in V2/V3 distribution trigeminal neuralgia (tic douloureux)
Unilateral throbbing pain migraine
Dull, bandlike or tightening pain tension
Sharp, stabbing pain esp. behind one eye cluster
N/V migraine, increased ICP d/t hydrocephalus or tumor
Photophobia, phonophobia, increased frequency of HA at menstruation migraine
Fever meningitis, local infection such as dental abscess
Unilateral lacrimation or rhinorrhea cluster
Primary Headaches
Primary HA Type
MaleFemale
Ratio
F>M
Age of
Onset
Type of Pain
Associated Si/Sx
Treatment
Teen
years
Unilateral or bilateral,
pulsing or throbbing
Tension
F=M
Any
age
Bilateral and occipital;
pain is dull and
bandlike, not throbbing
N/V, photophobia,
phonophobia
Aura is a transient
focal neuro sx
preceding HA; most
common type is
visual (flashing lights,
zigzag lines marching
across visual field);
develop over 5-20
min, and last 15 min1 hr; HA usually
follows w/in 20-60
min and lasts 4-72 hrs
Muscle spasm may be
involved
Cluster
M>>F
3rd
decade
of life
HAs occur in clusters in
which HA recur
cyclically (3-4x/day per
cluster) followed by
remission; each cluster
lasts up to several
months and remission
can be mo-yrs
Pain behind one eye or
over lateral part of nose
Prophylaxis: avoid
triggers, meds (betablockers like propranolol,
Ca++ channel blockers like
verapamil, TCAs like
amitriptyline, some
anticonvulsants like
depakote)
Abortive: simple
analgesics like
acetominophen, aspirin,
NSAIDS, ergot alkaloids
and selective 5-HT1
agonists like sumatriptan
Many of those used for
migraine
Also, PT, stress
management, biofeedback,
psychotherapy
Avoid possible precipitants
(e.g. EtOH, strenuous
exercise)
Verapamil for prophylaxis
Symptomatic tx includes
inhalation of pure oxygen
(>90% effective);
sumatriptan and
dihydroergotamine can be
used too
Migraine
(w/ aura= ”classic”)
(w/o aura=
”common”)
Ipsilateral
conjunctival injection,
lacrimation, nasal
congestion, and
occasional Horner’s
syndrome
Secondary HAs
HA Type
Subarachnoid
Hemorrhage
Etiology
Nontraumatic:
ruptured aneurysm,
bleeding from A-V
malformation
Description
Sudden, severe,
“worst HA of my
life”
Associated Si/Sx
LOC, vomiting,
neck stiffness
Diagnosis
LP or CT: look
for evidence of
hemorrhage or
heme b/d in
CSF
Temporal
Arteritis
(Giant Cell
Arteritis)
Subacute
granulomatous
inflammatory
condition involving
branches of external
carotid artery, esp.
temporal arteries
Occurs in elderly,
unilateral or
bilateral, over
temporal arteries
Scalp tenderness,
jaw pain during
chewing (jaw
claudication),
involvement of
ophthalmic artery
can lead to
blindness
Elevated ESR
to 100
Definitive dx
made by
temporal artery
biopsy
demonstrating
vasculitis
Trigeminal
Neuralgia (Tic
Douloureux)
Microvascular
compression of
trigeminal nerve
Idiopathic
Intracranial
HTN
(Pseudotumor
Cerebri)
A/w obesity
Facial pain
syndrome in which
brief severe
electrical shocklike pains occur in
distribution of a
branch of CNV (2
and 3 most
common); most
common in middle
age and elderly;
can be precipitated
by movement, a
cold breeze, or
tactile stimulation
in a trigger zone
Women in 2-4th
decades of life
Post-Lumbar
Puncture or
Low-Pressure
HA
Following LP
HA in an upright
position starting
w/in 48 hrs of LP
and resolving in
several days w/o
tx; HA relieved
when lying down
Treatment
Acute control of BP
for adequate brain
perfusion, monitoring
for vasospasm and
acute obstructive
hydrocephalus d/t
subarachnoid blood
Definitive tx:
surgical resection or
clipping of aneurysm
Prednisone in
decreasing doses for
several months and
then continued for
next 1-2 yrs
Carbamazepine
Bilateral visual
sxs: fleeting
loss of visual
acuity,
scotoma,
double vision,
perhaps
papilledema
CT/MRI
normal
LP shows
increased
pressure >250
mm water
Acetazolamide (CA
inhibitor): inhibits
CSF formation
Furosemide
Oral steroids
Serial LP to decrease
CSF pressure
If tx is ineffective, pts
may be left w/
permanent visual loss
IV caffeine sodium
benzoate
Blood patch