Download Disease/Pathophysiology Epidemiology Signs and Symptoms

Disease/Pathophysiology
Epidemiology
Signs and Symptoms
Migraine Headache
-Vascular and neurochemical disruption
(serotonin depletion)
-Cortical spreading depression decrease in blood flow begins occipitally
 vasoconstriction (aura) followed by
vasodilation and release of
inflammatory mediators
-Female predilection, first attack
often in childhood, genetic link
-Triggered by stress, hormones,
menstrual cycle, lack of sleep,
chocolate, red wine, cheese,
coffee)
-Recurrent moderate - severe h/a of variable
frequency in frontotemporal, ocular areas
-Usually unilateral (hemicrania)
-Prodrome: mood disturbances*, nausea, food
cravings
-20% of pts have an aura: dizziness, tinnitus,
photophobia, scotomas, hemianopsia, scintilla,
fortification, unilateral paresthesias or weakness
-Classic migraine (w/aura) vs. common migraine (no
aura) - 80%
-H/a is gradual onset by progressively intense throbbing, pulsatile
-Associated with N/V, photophobia, phonophobia,
dizziness, myalgia, vertigo
-Aggravated by physical activity and exertion
-Postdrome: food intolerance, impaired
concentration, fatigue, muscle soreness
-Risk of stroke x2-3 in migraine
pts, esp women on OC, aura
pts, migraine variants
Variants of migraine h/a:
Basilar migraine
-Vertebrobasilar vasoconstriction
Diagnosis
Treatment
-Analgesics/NSAIDS TOC for
mild/moderate h/a: aspirin, ibuprofen,
naproxen, ketorolac
-5HT1 receptor agonists (Triptans)
direct vasoconstrict cranial arteries,
can decrease headache of meningitis,
subarachnoid bleed
-Ergot
-Narcotics - Demerol
-Supportive: dark, quiet room,
withdrawal from stressful
surroundings, sleep, cold compress
-Prophylaxis: 1) TCA (amitryptyline),
beta-blockers, 2) CCB, relaxation
training, acupuncture
-Herbs: feverfew, ginger, cannabis
-Occipital h/a - aphasia, decreased hearing, vertigo,
tinnitus, ataxia, visual changes, N/V, dizziness,
bilateral paresthesias, LOC
-Transient unilateral hemiplegia/hemiparesis
-Ptosis, mydriasis
Hemiplegic migraine
Ophthalmoplegic migraine
-Palsy of ipsilateral CN III
Status migranosus
Migrainous stroke
-Persistent migraine, does not resolve on its own
-Neurological deficits persisting beyond migraine
attack - looks like ischemic stroke on CT
-Migraines >15 days/month for >6 months
Chronic migraine
Tension Headache
-Associated with muscular and
psychogenic factors
-MC chronic recurring h/a
syndrome
-Higher incidence in depression
-Episodic: associated with
stressful event - self limited
-Chronic: recurs daily, bilateral
-Gradual onset, bilateral bifrontal-occipitonucchal
location - band around the head, pressing, tight
-Worsens throughout the day
-Pain is mild - moderate, may occur under emotional
distress or worry
-No N/V, may have photophobia or phonophobia
-Muscular tightness and stiffness in neck, furrowed
brow, tense masseter muscles, poor posture
-May have tenderness in scalp or neck, insomnia,
teeth grinding, difficulty concentrating
Cluster Headache
-Hypothalamic hormonal influences,
pain @ level of pericarotid and
cavernous sinus complex
-Symp & parasymp input from
brainstem
-Least common, M>>F
-Triggered by stress, extreme
temp, alcohol, certain food,
altered sleep habits
-Episodic (MC) vs. cluster
-Excruciating, penetrating, piercing, stabbing,
exploding pain that begins after pt goes to bed
-Associated with ipsilateral lacrimation, miosis,
conjunctiva injection, nasal congestion, rhinorrhea,
ptosis, sweating, eyelid edema, Horner’s syndrome
-Acute attack: NSAIDs (naproxen,
ibuprofen, Tylenol, aspirin), triptans
-Prophylaxis: anti-depressants
-Supportive: relaxation, rest, massage,
heat packs, regular exercise, balanced
meals, spinal manipulation
DDX: ictal headache
-Acute: O2 100%, Samaritan DOC
-Prophylaxis: verapamil, ergots,
steroids
-Refractory: nerve blocks,
neurosurgery
Other: ice/heat, caffeine, water,
vigorous exercise/sex at onset
Headaches in general--
-Life threatening etiology symptoms: new
onset, change in character, progressively
intensifying, abnormal vital signs, fever, AMS,
abnormal neurological findings, seizures*,
meningismus*, DBP > 130, headache > 72 hours,
onset >55 y.o.
-DDX OF HEADACHE:
-Hypertensive h/a:
-Hypoxia-induced:
-Subarachnoid hemorrhage:
-Aneurysm/AVM:
-Meningitis/encephalitis:
-Subdural, epidural hematoma
-Brain tumor
-Trigeminal neuralgia
-Temporal arteritis
-Sinusitis
-Acute glaucoma
-Cervical spondylosis
-TMJ syndrome
-Rebound analgesic h/a
-Intracranial pathology
-Exertional headache
Traumatic Brain Injury
-Insult to the brain from external
mechanical force
-Systemic BP becomes determinant of
cerebral blood flow (hypotension fatal)
-Can be deceleration, acceleration,
coup, contrecoup
-Primary brain injury: initial structural
injury resulting from trauma
-Secondary brain injury: subsequent
injuries: hypotension, hypoxia, ^ICP
-M≫F, more common <35
-MCC MVA, Falls (>75),
firearms, battlefield explosions
-Also: sports related (boxing),
violence and suicidal behavior
Complications: focal neurological
deficits (MC CN I, IV, VII, VIII),
hydrocephalus, post-traumatic seizures,
vascular lesions, brain death, CSF
fistula
-Prevention: seatbelts/car seats,
don’t drink and drive, helmets,
fall precautions, firearm safety
Concussion
-Occipital, worse in the morning, pulsing/throbbing
-CO toxicity, sleep apnea, anemia
-H/a of sudden onset, vomiting, meningismus, AMS,
rupture of berry aneurysm, sentinel h/as
-Sudden onset, unilateral, severe, decreased vision
-Fever, non-focal neurological sx, meningismus, NV
-Pain on awakening, progressively worsens, worse
with Valsalva, ataxia, increased ICP, [h/a, vomiting,
papilledema], new onset seizures
-Transient, shock-like facial pain
-Elderly, severe unilateral scalp/temporal pain, PMR
-Stabbing/aching, rhinorrhea, worse forward
-N/V, eye pain, injection, increased IOP
-Posterior-occipital pain, increased by activity, h/o
trauma, spinal or muscle tenderness
-Temporal headache, earache, crepitus
-Overuse of analgesics - daily or near daily h/a
-Masses/lesions stretching arteries
-Benign h/a following cough, sneeze, Valsalva
-5 abnormal states of consciousness:
Stupor (unresponsive but arousable, sternal rub)
Coma (unresponsive and unarousable – short
duration)
Persistent vegetative (unconscious, unaware, have
sleep-wake cycles, reflex response)
Locked-in syndrome (pt aware, alert, paralyzed)
Brain death (lack of measurable brain function)
-Mild TBI: Conscious/LOC for few mins, dazed
feelings, h/a, confusion, dizziness, N/V, blurred
vision, tinnitus, fatigue, change in sleep, mood
changes, memory/concentration/attention change
-Moderate to severe: persistent h/a, vomiting,
seizures, unable to wake from sleep, pupil changes,
slurred speech, ataxia
-MC cognitive impairment: inability to form/store new
memory
-Brief LOC <30 min, transient confusion,
-ESR (r/o temporal
arteritis)
-Tests for underlying
condition (ABG, glucose)
-Head CT (r/o intracranial
masses, hemorrhage)
-LP (intracranial infx)
-Sinus imaging (r/o
sinusitis)
-MRI (r/o posterior fossa
lesion)
-CT without contrast, LP
-CT with IV contrast
-CT, LP with CSF analysis
1) CT, 2) MRI*
-Clinical
-Clinical, biopsy (giant cells,
increased ESR)
-Cervical spine XR, MRI for
herniated disc
-Neuro assessment with
GCS score, brainstem exam
(pupils, EOM, corneal/gag
reflex)
-CBC, BMP, PT, PTT,
Type/crossmatch
-Urine tox, blood alcohol
-Skull xray
-*CT w/o contrast
-MRI
-Angiography
-Carbamazepine
-Steroids (complications: blindness)
-Amoxicillin
-NSAIDs
-Wean them off
-Closed head injury: mild: observation,
supportive – return to ER if: severe
h/a, persistent N/V, seizures, confusion
or unusual behavior, watery discharge
from nose or ear
-Moderate risk: persistent emesis,
severe h/a, amnesia, LOC or
intoxication: observe & discharge
-High risk: intubate, send surgical
lesion to OR, fluid resuscitation
-Penetrating trauma: high-velocity
missile (bullets): debridement, dural
closure, skull reconstruction
-Penetrating object: removal,
angiogram, abx, tetanus vaccine,
debridement, wound irrigation
Complications: Second Impact
-Electrophysiologic dysfunction of
midbrain secondary to impact – no
evidence of structural alteration, no
fixed neurological deficits
Skull Fractures
-Can be linear (MC) or comminuted
-Depressed skull fx:
-Open skull fx:
-Basilar skull fx:
-Diastatic fx:
Epidural Hematoma
-Between skull and dura mater:
laceration of middle meningeal artery
 rapid brain compression
-Arterial bleed, enlarges rapidly
Subdural Hematoma
-Bt dura mater and brain – mov’t of
brain relative of skull leads to rupture
of bridging vessels – venous bleed
Subarachnoid Hemorrhage
-Intraparenchymal bleed: saccular
aneurysms @ bifurcation of arteries and
circle of Willis that rupture, AVM
Diffuse Axonal Injury
-Neuronal injury in the subcortical gray
matter or the brainstem from severe
rotation or deceleration
Bacterial Meningitis
Inflammation of the leptomeninges and
underlying CSF - transmitted through
hematogenous spread (resp tract MC),
direct extension (nasopharynx), direct
inoculate (trauma), neurosurgery
-Increased permeability of BBB 
cerebral edema  toxic mediators in
CSF  decreased perfusion
-Risk: Head injury with skull
fracture
Viral (Aseptic) Meningitis
-Transmission is hematogenous* or
neural penetration (Herpes)
-Mostly caused by enter viruses,
coxsackievirus B in children <3
disorientation, dizziness, impaired consciousness,
memory loss, irritability, problems with
concentration/seizure on impact
-GCS >13
-Post-concussive syndrome: h/a, N/V, memory loss,
dizziness, blurred vision, emotional lability*, sleep
disturbances*  do not confuse with PTSD
syndrome: Pt suffers another brain
injury before first is resolved  fatal
-Comminuted fracture displaced inwardly
-Tx surgically only if segment is
depressed >5mm on CT
-Laceration over skull fracture, involving ear/sinus
-Linear fx @ skull base with CSF otorrhea, rhinorrhea, hemotypanum, battle sign, raccoon eyes
-Linear fx that causes bones of skull to separate @
unfused sutures
-Brief LOC  lucid period  rapid progression to
unconsciousness
-Ipsilateral dilated pupil from uncal herniation,
contralateral hemiparesis
-CT: lenticular biconvex
mass
-Burr hole
-Risk: blunt head trauma,
alcoholic, elderly  brain
atrophy
-Acute or sub-acute, unconsciousness on impact 
increased ICP. Gradual decline in mental status and
change in LOC, h/a, cortical dysfunction
-CT: crescent shaped
concave hematoma – less
dense than epidural
-Trephination (burr hole), emergent
craniotomy if refractory
-MCC rupture of aneurysm
-Sudden, severe h/a without focal neurological sx,
vomiting, retinal hemorrhage, meningeal irritation,
nuchal rigidity, photophobia
-Surgery, clip aneurysm, analgesia,
stool softener, IV fluids, anti-HTN
-Rarely causes death, 90% of
pts remain in persistent
vegetative state
-Shearing: breakdown of overall communication
among neurons in the brain
-Severely depressed consciousness, neurological
dysfunction or coma
-Prodromal URI symptoms
-**Headache, meningeal irritation (nuchal rigidity,
Kernig sign, Brudzinski sign), high fever and chills,
photophobia, vomiting
-Papilledema, seizures, focal neurologic sx, AMS
-N. meningitides: nonblanching petechiae and
cutaneous hemorrhages
-Infants: lethargy, change in level of alertness, poor
feeding, vomiting, resp distress, apnea, cyanosis,
bulging fontanelles
-Constitutional symptoms then high grade fever
-Severe headache, meningismus, photophobia
-Occasional AMS, seizures
-Viral syndromes: pharyngitis, zoster, viral
exanthems
-CT w/o contrast (do a LP if
CT is negative and
suspicion high –
xanthochromia)
-May have normal CT,
show punctuate
hemorrhages, ICP in
reference range
-CBC-D, electrolytes,
glucose
-BUN/Cr, LFTs
-Blood, nasopharynx, resp,
urine cultures
-Head CT with contrast
-*Lumbar puncture
-Head CT with/without
contrast
-LP with CSF analysis
-Cultures, EEG
-Supportive: rest, hydration,
antipyretics
-Herpetic infx: IV acyclovir early
-HIV meningoencephalitis: HAART
-CMV infx: IV ganciclovir
-Age <5, >60,
immunosuppression, *crowding
(dorms), splenectomy
(See Chart on pg 5)
-Administer abx/steroids within 30
mins of presentation - empiric therapy
if LP cannot be performed
-Manage fever and pain, elevate pain,
hyperventilation
+/- Dexamethasone
-Prophylaxis: rifampin, cipro,
ceftriaxone
-Prevention: MCV4 vaccine
Fungal Meningitis
-Opportunistic infx in
immunocompromised pts
-Cryptococcus neoformans*,
coccidioides immitis, candida
albicans, histoplasma
capsulatum, blastomyces
Noninfectious Meningitis
-Metastasis to the meninges, drugs
(NSAIDs, abx, IV Igs), inflammatory
conditions (sarcoidosis, SLE, etc)
No associated organism
Encephalitis
-Viral infx  inflammation of brain
parenchyma with neuropsychological
dysfunction
-Transmission human-human or
reactivation of HSV
-Virus gains entry to CNS by
hematogenous spread, traveling along
neural and olfactory pathways
-Inflam. diffusely affects gray matter
-*Arboviruses - MCC - West
Nile, *St Louis (urban areas
around the Mississippi),
*California virus (northern
Midwest, east), EEE (deadliest),
WEE, JE (MC internationally)
-HSE - MC in neonates
-VZE in immunocompromised
Brain Abscess
-Caused by intracranial inflammation
with subsequent abscess formation
-Transmission: contiguous spread *sinusitis, otitis media, dental infx,
mastoiditis; hematogenous spread systemic infx (endocarditis, lung infx,
skin infx, IVDA, HIV); trauma
Multiple Sclerosis
-Autoimmune idiopathic inflammatory
demyelinating CNS disease
-Destroys myelin sheath, spares axons,
neuronal cell bodies
-Autoantibodies to myelin basic protein
in blood and CSF
-Inflammation and demyelination with
remyelination  lesions evolve into
plaques, axonal transections 
permanent disability
-Selective for optic nerve, white matter,
brain stem, basal ganglia
Complications: coma, delirium,
parapleglia, UTIs, blindness,
complications from chronic disability
Prognosis: normal life span, decreased
quality of life
-Culture is diagnostic TOC
(India ink)
-Detecting capsular Ag in
CSF
-Mild: fluconazole
-Severe: amphotericin B
-Viral prodrome: fever, h/a, N/V, lethargy, myalgia
-Encephalopathy: *acute confusion or amnestic
states, behavioral and personality changes, stiff
neck, photophobia, lethargy, decreased LOC, seizure,
ataxia, flaccid paralysis, cranial nerve deficits,
meningismus
-Herpetic skin lesions in neonates*
-Viral serology (arbovirus)
-CSF PCR
-Head CT with and without
contrast
-MRI, EEG
-Brain biopsy
-Supportive: manage fever and pain,
control straining, coughing
-Elevate head, hyperventilation
-If ICP: diuresis, dexamethasone
-HSV meningoencephalitis and VZV
encephalitis: *IV acyclovir
-HIV pts: IV foscarnet
-*S. aureus, *streptococci,
*pseudomonas
-Secondary to size and location of the abscess:
-Cerebellar - nystagmus, ataxia, dysmetria;
brainstem - facial weakness, dysphagia,
hemiparesis; frontal - inattention, drowsiness, AMS,
motor speech disorder, hemiparesis, grand mal
seizures; temporal - ipsilateral aphasia, visual
defects
-[Fever, localized headache, focal neurological
deficit]
-Mental status changes, seizures, N/V, nuchal rigidity
-*Sudden worsening h/a + meningismus = rupture
-Moderate leukocytosis,
increased ESR and CRP,
blood cultures
-CT-guided needle
aspiration
-LP nonspecific
-*CT with contrast - “ring
enhancing lesion”
-MRI more sensitive
-MC debilitating disease in
young adults in US
-F>M, Caucasians > other,
north European descent
>2 episodes of sx + > signs (clinical/ imaging) that
reflect dz in noncontiguous white matter in CNS::
-*Sensory loss MC (transient) - decreased vibration,
joint position
-Motor dysfunction - plasticity, muscle cramping,
weakness, paralysis, hyperreflexia, Babinski, UMN
dysfunction, seizures, aphasia, dysphagia
-Autonomic dysf. - bowel, bladder, sexual
dysfunction, incontinence, constipation
-Cerebellar sx - dysarthria, disequilibria, trunk/limb
ataxia, tremor
-Constitutional - fatigue MC (esp after hot
shower/activity), weight loss, dizziness, joint pain
-Mental status - difficulty concentration, memory,
judgment, emotional lability, depression
-Trigeminal neuralgia - transient shock
-Lhermitte sign - flexion of neck = shock-like feeling
in torso or extremities
-CBC-D, glucose, UA,
culture, electrolytes (r/o
electrolyte disturbance)
-Head CT to assess focal
neurological or mental
status changes
-*MRI of head with
gadolinium: appear as
plaques with periventricular distribution, #
of plaques not proportional
to severity
-MRI of spine with
gadolinium for pts with
acute transverse myelitis
-CSF analysis - elevated
protein, oligoclonal bands,
normal glucose, increased
MBP
-Abscesses <2.5 cm = abx
-Surgical excision/drainage + 6-8
weeks of abx is the mainstay of tx
-Strep: PCN G, 3gen CPN
-S. aureus: nafcillin, vanco
-Pseudomonas: cefipime, ceftazidime
-H flu, s. pneumo - chloramphenicol
-Multiple abscesses or abscess in
essential brain area: repeated
aspirations with high dose abx instead
of complete excision
+/- Corticosteroids
-Acute optic neuritis, general MS
exacerbations: *IV methylprednisolone
(Solu-Medrol)
-Acute trans. myelitis, encephalitis
exacerbations: IV dexamethasone
-RR-MS: B-interferon, glatiramer
acetate (synthetic MBP)
-Natalizumab (Tysabri) - blocks
attachment of immune cells to BB
vessels - final line treatment
-Progressive disease or relapse
prevention: immunosuppressants
-Constipation: stool softeners, bulk
producing agents, laxative
suppositories
-Spasticity: baclofen, diazepam
-Depression, lability: amitriptyline
-Supportive: self-catheterization, skin
care
Categories:
Relapsing-remitting: acute
exacerbations (usually previous
sx) with gradual remissions starts ages 20-40
Primary progressive: MC
after age 40, gradual decline,
accumulation w/o remission
Secondary progressive: 50%
RR-MS enter ~ 10 years
Progressive relapsing: PP-MS
with sudden episodes of new
symptoms or worsened existing
ones
Idiopathic inflammatory
demyelination syndromes
-May be unrelated to MS, a
presenting symptom or
exacerbation
Optic neuritis:
Bilateral internuclear ophthalmoplegia:
Acute transverse myelitis:
Inflammation/demyelination of optic nerve - acute
onset (unilateral visual blurring, flashes of light,
central scotoma, decreased acuity & color
perception, discomfort) - Uhthoff phenomenon
(visual deterioration induced by heat)
-MLF coordinates CN III, IV, VI,
VIII - direction of eyes based on
equilibrium
Devic syndrome:
Acute disseminated encephalitis:
Amyotrophic Lateral Sclerosis
-Degenerative neuromuscular dz
affecting both UMN and LMN - only
MOTOR neurons affected
-Familial: genetically transmitted
-Sporadic (90%): idiopathic, elevated
levels of glutamate in serum and CSF nerve cell degeneration, grouped
muscle atrophy
Complications: pneumonia, decubitus
ulcers, DVT/PE, UTI, sepsis
-M>>F, Whites > other
-Onset at age 40 - 60
DDX:
-UMN lesions (brainstem
lesions, stroke, demyelination)
-LMN lesions (CN palsies, spinal
cord trauma, tumor,
radiculopathy, neuropathy)
-Guillain-Barre
-Peripheral nerve lesion (ALS
lacks pain and sensory sx)
Lesion in MLF leads to adduction deficit in each eye,
nystagmus upon adduction
Lesions in spinal cord - acute partial loss of motor,
sensory, autonomic, reflex, sphincter function below
level of lesion
Acute transverse myelitis + bilateral optic neuritis
Similar to MS: acute onset of motor, sensory,
cerebella and CN defects with encephalopathy, AMS
 coma, death
Combinations of:
-Difficulty swallowing, facial paresis - drooling,
slurred speech, dysarthria
-Bilateral limb weakness + atrophy - early sx:
clumsiness, twitching, fasiculations, cramping,
weakness; wrist/foot drop, increased tone (UMN)
-LMN - muscle fasciculation, esp tongue
-Unexplained weight loss - cachexia
-Pseudobulbar effect: pathological laughing/crying
-Bulbar ALS: dysphagia, drooling, dysarthria
-Affect resp muscles and vocal cords late ventilation, gastrostomy
-Classic signs: asymmetric muscle weakness,
increased tone, hyperreflexia, fasciculation, muscle
atrophy
-MRI of brain and spinal
cord - r/o differentials
-EMG: increased amplitude,
long duration
-Muscle biopsy: grouped
atrophy
-FVC: <50% predicted is
indicator of advanced
disease
IV steroids, seizure prophylaxis,
plasmapheresis for antibodies
-Riluzole (rilutek) - blocks glutamate,
prolongs need for tracheotomy
-Baclofen (lioresal) - treats spasticity
-Focus on respiratory sx, control of
secretions: amitriptyline
-Ventilatory support for resp failure
-Sedation and pain control
-Constipation: laxatives, stool
softeners, dietary changes
-PEG placement
-Median survival 3 - 5 years after dx,
hypoxia or cardiac arrythmias MCC
death in pts not on ventilator;
pulmonary infx MCC death in pts on
ventilators
Seizures
-Abnormal, excessive excitation of
cortical neurons (seizure focus) sudden change in electrical activity
(motor, sensory, behavioral changes)
with/without change in consciousness
-Manifestation depends on location and
extent of transmission: temporal lobe taste, smell, psychic phenomena;
occipital - visual; parietal - sensory
-Epilepsy: 2+ recurrent idiopathic
seizures
-10% likelihood of having at
least 1 seizure in lifetime
-3% chance dx of epilepsy
-Epilepsy highest in ages <2
and >65
-Metabolic/electrolyte
disturbances:
hypo/hyperglycemia, Na & Ca
disorders, uremia, thyroid
storm, hyperthermia
-Mass lesions: brain tumor,
metastasis
-Missing drugs: non-compliance
anti-epileptics (MCC),
alcohol/drug withdrawl
-Misc.: eclampsia, HTN
encephalopathy, genetics,
environmental
-Injury: prenatal/birth, hypoxia,
head injury
-Ischemia: CVA, TIA, ICP
-Infx: sepsis, brain abscess,
meningitis, encephalitis
-Intoxication: cocaine, Li,
lidocaine, metals
-Associated s/s: fever, focal
neurological findings, head
trauma with papilledema,
meningismus, needle tracks, h/a
4. Febrile seizures: generalized
seizure occurring 3 mo - 5 years
old
-Lasts <15 min
-Pt has fever w/o evidence of
any other cause for seizure
-Complex febrile seizures:
multiple seizures < 24 hr, focal
or prolonged seizure
-First febrile seizure should
receive fever workup: CBC,
culture, UA, CXR, LP
1. Partial - seizure focus in one hemicrania:

Simple: brief sensory, motor, autonomic or
psychic change (paresthesias, muscle spasm,
mood changes, hallucination) - no LOC Jacksonian seizures: involve primary motor
cortex, temporary weakness afterward

Complex: behavioral arest x 60-90 sec  brief
post-ictal confusion - automatisms - pt is
unresponsive to verbal command, resistant to
physical manipulation - mostly originate in
temporal lobe (bizarre motor behaviors) precedent aura (odor/taste) which may be
simple partial seizure - followed by h/a

Secondarily generalized - begins with aura,
evolves into complex partial seizure 
generalized tonic-clonic seizure
2. Generalized - affects both cerebral hemispheres,
always results in LOC

Absence (petite mal): brief episodes of impaired
consciousness, sudden immobility and blank
stare - onset in childhood - no convulsive
activity/aura, few automatisms (repetitive
blinking) - precipitated by hyperventilation,
photic stimulation - EEG shows 2.5 Hz
generalized spike and slow wave complexes

Myoclonic - sudden, brief, repetitive, arrhythmic
jerking movements <1 seconds - twitch/jerk localized to few muscle groups - EEG: fast
polyspike-and-slow wave complexes

Atonic - sudden loss of postural tone, pt drops
to the ground (r/o stroke, vasovagal syncope)

Tonic-clonic (grand mal) - sudden onset of
bilateral, symmetric contraction for several
seconds, pt falls, clonic rhythmic movements
(perioral cyanosis)  flaccid with prolonged
postictal confusion - preceded by auras,
impaired memory of event, urinary/bowel
incontinence, posterior shoulder dislocation Todd’s paralysis
3. Status epilepticus - seizures > 30 min, repetitive
generalized seizures w/o return to consciousness

Autonomic phenomena (piloerection, mydriasis,
prolonged apnea), metabolic changes (lactic
acidosis, CO2 narcosis, hypekalemia,
hypoglycemia), HTN, arrhythmias, increased
secretions, airway obstruction/aspiration, acute
tubular necrosis, focal ischemia in brain postictal: fever, tachycardia, mydriasis,
decreased corneal reflex, positive Babinski
-New onset seizure: CBC,
electrolytes, glucose, LFTs,
UA, drug screen, EEG, head
CT, search for underlying
cause
-Chronic seizure disorder
with pattern: glucose/ anticonvulsant levels
-Anti-convulsant levels:
inadequate medication MCC
of recurrent seizures
-EEG: negative EEG does
not r/o seizure disorder often need repetitive EEG,
may be normal between
seizures - neuron focus
shows as a spike - if
abnormal electricity <1000
neurons, there will be no
positive findings
-Non-contrast head CT
-MRI - sensitive for lowgrade tumors, vascular
lesions, inflammation, CVA
-Neuro consult
-Single unprovked seizure: avoid
precipitations, no anticonvulsants
recommended
-Risk of recurrence depends on 1)
abnormal brain MRI, 2) abnormal EEG
-Benzos for actively seizing pts,
phenytoin or Phenobarbital 2nd line
-Absence seizures: *ethosuximide
-Myoclonic: valproic acid, aborigine,
torpiramate
-Tonic-clonic: valproic acid,
carbamazepine, phenytoin
-Partial: *carbamazepine, phenytoin,
Phenobarbital
-Ketogenic diet
-Pregnancy: folic acid 1 mg/day - do
not switch medication, use only 1
anticonvulsant - obtain frequent drug
serum levels
-Surgery: vagus nerve stimulation,
partial/full resection of focus,
disconnection procedure (corpus
callostomy)
-Trauma common in tonic-clonic
seizures: ecchymosed, abrasions,
tongue/limb lacerations
-MC serious injury associated with
epileptic seizures: burns
-During seizure, don’t put anything in
pt’s mouth, put something under their
head, take off constricting clothing, do
not restrain, roll to the side
Bell’s Palsy
-Interruption of CN VII
-May be due to trauma, surgical
intervention, tumor, stroke,
infection of CN VII
-MCC is idiopathic
-Attacks pregnant women,
people with diabetes, influenza,
URI
-Can be caused by HSV, Lyme
disease, sarcoidosis
-Unilateral, acute onset
-Paralysis of muscles of facial expression: corner of
mouth droops, crease and skin folds effaced (loss of
wrinkles), forehead unfurrowed, eyelids do not close,
drooling, heaviness/numbness in face, retroaurical
pain, hypersensitivity to sound in affected ear,
impairment of taste (dysguesia)
-Edema
DDX: acoustic neuronal
(Schwannoma) - no
resolution, worsening of sx
+ tinnitus, vertigo; stroke;
intracranial mass
-Steroids
*Acyclovir + prednisone
-Intraocular saline to prevent drying of
eye
-Analgesic for pain relief
-Massage of weakened muscles
-Splint to prevent drooping of lower
part of face
Neuropathic pain
-Direct injury to nerves
Mononeuropathy: injury or damage of
isolated nerve - caused by trauma and
local compressive factors
-Diffuse polyneuropathies are
symmetric, initially distal, complication
of systemic disease
Radiculopathy: irritation and
compression of nerve root of the spinal
cord  numbness, tingling,
paresthesias, weakness of
corresponding dermatome
-Mononeuritis Multiplex: motor and
sensory, affects 2+ nerve distributions MC associated with DM and multiple
nerve compression (RA) - initial
asymmetric random distribution 
progressive course
-Complex Regional Pain Syndrome
- severe, burning neuropathic pain in at
least one limb, increased at night precipitated by trauma - ass/with
alloying, hyperalgesia, extremity
edema, vascular ischemia, osteopenia
-DM: diabetic neuropathy
-Alcoholism: thiamine,
cyanocbalamine deficiency,
buildup of toxic metabolites
-Hypothyroid, liver failure, renal
insufficiency, drug induced,
environmental (toxic), radiation
exposure
-Trauma, complex regional pain
syndrome, infectious,
neoplastic, sarcoidosis, RA,
collagen vascular disease,
porphyria, Guillain-Barre,
neurofibromatosis, CharcotMarie-Tooth disease, unknown
-Nerve entrapment (carpal
tunnel), nerve compression
(tumors, cysts, herniated disc),
neuralgia (trigeminal, postherpetic)
-Pain - shooting, radiating, unilateral and of distal
extremities initially, esp pronounced at night,
alloying/hyperesthesia
-Associated with numbness, paresthesias, tingling,
sensory deficits, weakness, paralysis, nerve palsies,
muscle spasm and pain, dizziness/impotence
(autonomic neuropathy), balance and coordination
deficits
-On PE: Orthostatis hypotension, cranial nerve
deficits, peripheral nerve deficits (foot drop - inability
to dorsiflex the foot), muscular atrophy, muscle
tenderness, hyporeflexia, weakness, sensory deficits
(vibratory, temp, pain), gait disturbances, skin
findings (ulcers, gangrene, Raynaud’s phenom, café
au lait), charcot’s joint
-Autonomic: anhidrosis, pruritis, bladder atony
(retention), impotence, uncreative pupils, postural
hypotension, dizziness, syncope, heart block, **silent
MI, gastric atony, diarrhea, constipation, fecal
incontinence, impaired hypoglycemia awareness
-CBC-D (infx/anemia)
-BMP - electrolyte abn.
-ESR, Crp
-Rhematoid factor (ANA)
-Serology (infx)
-Serum protein
electrophoresis (multiple
myeloma)
-Thyroid panel
-Liver/hep. Panel
-ALP
-Vitamins (B1, B6, B12)
-CSF
-CT, MRI, x-ray
-Cultures
-UA - heavy metal tox
-Nerve bx
-NCV/EMG
-Underlying disorder
-Neuropathy management amitriptyline, nortriptyline, mexiletine,
gabapentin, carbamazepine,
phenytoin, aborigine, topical lidocaine,
baclofen, lyrica
-Analgesics - NSADIs, SSRIs, opioids
-Muscle relaxants
-Remove offending agent
-Steroids
-Cytotoxic agents
-Plasmapheresis
-Supportive (TENS, massage, hypnosis,
meditation, acupuncture)
-Autonomic neuropathy: clonidine,
midodrine, fludrocortisone,
bethanechol, compression stockings,
symptomatic
-Physical activity/therapy
-Surgery
Primary Brain Tumors
-Ionizing radiation
-Immunosuppression
-Inherited: Neurofibromatosis
(gene 17), Bilateral vestibular
schwannomas (chromosome
22q)
-H/o cancer
-Sx depend on location: asymptomatic, systemic
(weight loss, malaise, anorexia, fever), mental status
changes (emotional lability, personality, intellectual
decline, depersonalization, memory loss), new-onset
seizures, headache (ipsilateral, early, waking pt from
sleep, relieved with head elevation, worsened by
coughing, bending), hallucinations, visual
disturbances, hearing loss, vertigo, tinnitus, N/V, CN
deficits, gait abnormal
-PE: papilledema, CN deficits (nystagmus, EOM
dysfunction) hydrocephalus (MC in ependymoma),
contralat motor/sensory deficiencies, weakness,
hypotonia, AMS, memory def., ataxia, UMN def.
(hyperreflexia, spasticity, Babinski+, Romberg+,
pronator drift+, atrophy, Hoffman reflex
DDX: CVA, subdural
hematoma, tuberculoma,
brain abscess,
toxoplasmosis, Bell’s Palsy
-CBC-D, BMP, Skull XR, CT,
*MRI, EEG, LP, PET< MRA,
Bx
-Corticosteroids (dexmethasone),
-Osmotic diuretic: mannitol
-Anticonvulsants
-Surgical excision TOC
-Ventricular shunting (conduit for CSF)
-Radiation (gamma knife)
-Chemo (tx underlying malignancy –
intrathecal)
-Palliative
-Referrals
-Meningioma: benign tumor derived
-Common in adults, middle
Complications: herniation, brain
abscess, progressive focal/global
motorsensory abnormalities
-Surgery, external beam, particle
radiation
from arachoid, attaches to dura
-Acoustic neuroma: Schawnn cells of
CN8 and 5, as/with neurofibromatosis
type 2
-Pituitary adenoma: as/with increased
hormone secretion
-Metastatic carcinoma
-Craniopharyngioma: benign brain
tumor – Rathke’s pouch
-Germinoma: pineal, 3rd ventricle
-Dermoid cyst: benign, midline
supratentorial/cerebellopontine angle
-Epidermoid tumor: benign cystic
-Primary cerebral lymphoma: B-cell
malignancy as/with EBV
-Glioblastoma multiforme: Grade IV
astrocytoma
-Astrocytoma: located in cerebrum,
cerebellum, brain stem, spinal cord
-Ependymoma: derived from 4th
ventricle epithelium
-Medulloblastoma: post fossa, 4 vent.
-Oligodendrocytoma
Metastatic Tumors
-Lung
-Breast
-Melanoma
-Others: GU (Renal Cell Ca), osteosarcoma, head/neck, lymphoma (NHL)
Ischemic Stroke
Large Vessel Thrombotic Stroke:
thrombi from atherosclerotic plaques at
arterial bifurcations
Small Vessel Stroke Lacunar Infarct:
occlusion of small branches of large
cerebral arteries (leave behind lacunae)
Cardiogenic Embolic Stroke: thrombus
from heart to brain - MCA
Hemorrhagic Stroke
-Rupture of vessel  hemorrhage
aged, F>M
-More common than primary
tumors, ages 35-70
-MC in 20s
-MC adult primary intracranial
neoplasm
-Translabyrinthine surgery,
radiosurgery for larger tumors
-Progressive ipsilateral unilateral sensorineural
hearing loss, tinnitus, vertigo, facial
weakness/numbness
-Bitemporal hemianopsia, hormone secreted
(prolactin, FSH/LH, GH, ACTH, TSH)
-May be 1st sign of systemic cancer or have no
primary cancer site
-Visual and endocrine disturbances
-May be benign or aggressive  causes DI
-Entrapped skin tissue during closure of neural tube
-Arise from embryonic epidermal tissue
-Most clinically aggressive tumor, median survival 512 mo
-Common adult tumor, follows protracted course
-Transsphenoidal excisional surgery,
appropriate medical treatment
-Surgery, focal radiation
-Surgery
-CT/MRI: ring-enhancing
lesions
-Surgery (often not resectable)
-In adults, found in lumbosacral spinal cord
-Surgery, external beam radiation
-Slow, benign course
-Surgery, chemoradiotherapy
-Surgery preferred
-MC source of brain metastasis
-Metastasis to brain or skull but not both
-Most likely to metastasize
-Palliative
-Glucocorticoids, antiseizure meds,
surgery, *whole brain irradiation,
chemotherapy, gene/immunotherapy
-Common in children <20 yrs
-MC brain tumor in children
-In children, primary malignant
tumors 2nd MCC of cancer death
(MCC leukemia)
-MC type found in large vessels
of brain
-Rheumatic heart dz, Afib, MI,
vent. aneurysm, endocarditis
-Risks: HTN, age, brain tumor,
AV malformation
-Surgery
-Glucocorticoids, high dose
methotrexate/cytarabine, irradiation
-MCA: MC – facial droop, arm weakness,
hyporeflexia, contra. Hemiplegia/ hemiparesis to face
& arm, homonymous hemianopsia of contralateral
eye, confusion, global aphasia, apraxia; ACA: contra.
hemiplegia to foot or leg, impaired gait, hemiparesis
to toes, foot, leg, difficulty decision making, urinary
incontinence, cognitive disorders; PCA:
homonymous hemianopsia of contra. visual field, loss
of central vision, memory deficits, anomic aphasia,
alexia; basilar/vertebral: visual disturbances,
dysphagia, N/V, ipsilateral Horner’s, ipsilateral loss of
facial pain and temp sensation
-Pure contralateral motor hemiplegia (face, arm, leg)
OR pure sensory (transient numbness, sensory loss
to face, arm, leg), ataxic hemiparesis: weakness of
lower limb, babinksi +, dysarthria
-Neuro deficit with cardiac condition
-History/PE/Neuro
-CT, MRI, perfusion scans,
arteriography, MRI
angiography
-MRI
-DDX: tumors, infx,
neurosyphilis, drug use
-CT, carotid Doppler and
US, cardio work-up
FOR ALL:
-Anticoagulation with heparin,
antiplatelet therapy, whole brain may
be salvaged with TPA (penumbra)
-PT, OT, ST, prevention of secondary
complications (DVT)
Compl.: reoccurrence, increased ICP,
vasospasm, hydrocephalus,
hypothalamic dysfnctn
-Cerebral aneurysm: Berry aneurysm
AVM: Congenitally abnormal arteries
and veins – lack of capillaries =
increased pressure in veins
Locked-In Syndrome
-Brain stem stroke – ventral part of
pons damaged, no upper brain damage
Uncal Transtentorial Herniation
-Herniation often due to trauma to
lateral side of head
Central Transtentorial Herniation
-Downward displacement of thalamic
region – gradual compression of
brainstem
Tonsillar Herniation –cerebral
hemispheres compress medulla through
foramen magnum
Infratentorial Herniation – can be
upward (hydrocephalus/coma) or
downward (cardiac arrest/death)
Increased ICP
-Normal ICP 0=15 mmHg, elevated ICP
compromises cerebral vessel
autoregulation diminished perfusion
 ischemia of cerebral tissue 
vasodilation  further increased ICP
-PCKD, coarctation, AVM, HTN,
atherosclerosis
-TBI, medication OD, damage to
myelin sheath
-Cognitively intact but quadriplegic
-Able to sense pain and touch
-Supratentorial lesions
-Compression of CN3: Ipsilateral pupil enlargement,
sluggish pupil response, neurolenic hyperventilation,
contralateral hemiparesis (due to pyramidal tract
compression), brainstem compression, HTN and
bradycardia (Cushing’s)
-Midpoint, non-reactive pupils, decorticate 
decerebrate, Cheyne-Stokes respiration, Babinski+,
Cushing’s triad (HTN, bradycardia, irregular
respirations)
-Compress cardiovascular, resp centers  cardiac
arrest
-Brainstem dysfunction (ataxia), CN palsies (absent
corneal, gag reflex), limb weakness or sensory loss
prior to nonreactive pupils, abnormal respirations
-Earliest signs: headache, AMS, confusion,
drowsiness, decreased LOC, Cushing’s reflex
-Cushing’s triad, bilateral fixed dilated pupils
Psychogenic Coma
Brain Death
-Cessation of all brain function
Cerebral Palsy
-Neurological disorders caused by
*nonprogressive brain lesions involving
motor or postural abnormalities noted
in early development
-Lesions restricted to brain only
-Spastic CP: occurs at term – vascular
injuries  spastic hemiplegia;
hypoperfusion  spastic quadriplegia
-Diskinetic CP: hypoperfusion to basal
ganglia
-Chronic h/a, N/V/dizziness, neurologic deficits
Rupture leads to subarachnoid hemorrhage: severe
and sudden generalized h/a which subsides after 1-2
weeks, HTN, collapse and LOC, vomiting, nuchal
rigidity, photophobia, fever, coma, focal
motor/sensory deficits, cranial nerve deficits
-SAH, seizures*, throbbing severe h/a, hemiparesis,
speech deficits, learning disorders
-Age of onset: fetal/neonatal
-Maternal and prenatal RFs:
long menstrual cycle, previous
pregnancy loss, maternal MR,
thyroid disorder, seizure
disorder
-Pregnancy RF: polyhydramnios,
tx of mother with thyroid meds
or hormones, HTN, mercury
exposure, congenital
malformations, male sex, 3rd
trimester bleeding, IUGR
-Cushing’s reflex: HTN, bradycardia, widened pulse
pressure
-Cushing’s triad: HTN, bradycardia, irregular
respirations
Resistance to having eyelids opened, nystagmus on
caloric testing, adverse head/eye mov’ts, failure of
pts arm to fall on face when released
-Pupillary response absent (dilanted), corneal/gag
reflex absent, no facial or tongue mov’t, flaccid limbs
-Spastic (MC): spasticity, hyperreflexia, clonus
Babinski+

Spastic hemiplegia: one side of the body
with UE spasticity > LE spas., normal IQ,
one-sided UMN deficit, specific learning
disabilities, seizures

Spastic diplegia: involvement of bilateral
LE > UE, normal IQ, UMN deficits,
scissoring gait

Spastic Quadriplegia: all four extremities,
associated with mental retardation
-Dyskinetic: extrapyramidal signs characterized by
-CT, CSF – elevated
opening pressure,
xnathochromia; cerebral
angiography
-Cerebral arteriography within 24-72
hrs to localize bleed
-Surgery, supportive (prevent increase
in ICP), pt education, management of
HTN
-Cerebral angiography
-Surgical, endovascular occlusion
-Neuromuscular stimulation, supportive
-CT w/o contrast
-EEG, brain scan: no
cerebral blood flow
-Clinical
-Coagulation profile (r/o
cerebral infarction)
-Screen for metabolic or
genetic disorder:
lactate/pyruvate, thyroid
function, ammonia, serum
quantitative amino acid &
urine quantitative organic
values, chromosome
analysis
-Cranial US
-Hyperventilation, intubation, mannitol,
narcotics (sedation), ventricular
catheter for ICP, glucocorticoids
-Reverse Trendelenburg position (not if
hypotensive, spinal injury) – venous
outflow improved, decompressive
craniectomy – large section of skull
removed, ventriculostomy
-Screen for MR, visual and hearing
impairments, speech and languae
disorders, oromotor dysfunction
-Balcofen and benzos for spasticity
-Antiparkinsonism drugs,
anticonvulsants (diazepam, valproic
acid, barbiturates)
Consults:
-Physiatrist: phenol IM neurolysis,
botox injx to reduce spasticity
-Orthopedist: surgical management of
-Perinatal RF: Prematurity,
chorioamnionitis, nonvertex and
face presentation, birth
asphyxia
-Postnatal RF: Infx, ICH,
hypoxia-ischemia, kernicterus,
persistent fetal circulation, low
APGAR
abnormal mov’ts, hypertonicity, early hypotonia with
mov’t disorder @ 1-3 yrs, DTRs normal
-Mixed: no specific predominant tonal quality
-Hypotonic (rare): truncal and extremity hypotonia
with hyperreflexia, primitive reflexes
-CT of brain
-MRI of brain*
-EEG, EMG, NCV, histology
-Infant presents with early hypotonia for first 6
months, followed by spasticity
-No race predilection, M>F
Muscular Dystrophy
-X linked recessive disorders with
progressive proximal muscle weakness
caused by muscle fiber degeneration:
mutation of xp21 locus (dystrophin)
Duchenne Muscular Dystrophy
-Severe absence of dystrophin (found in
skeletal and cardiac muscle) – repeated
cycles of necrosis in response to trauma
-Most severe form
-Progressive, symmetric weakness that
affects proximal muscles, initially in
lower limbs (pelvic girdle)
Becker Muscular Dystrophy
-Production of abnormal or less
dystrophin
Mytonic Dystrophy
-Autosomal dominant – mutations in
DM1 (more common) and DM2
Limb-Girdle Dystrophy
-MC
-Only males affected –
manifests between ages 2-3
-Calf pseudohypertrophy (fatty, fibrous replacement
of muscle) – false appearance of healthy muscle
-Frequent falls, difficulty running, jumping, climbing
stairs, rising from the floor
-Toe walking, waddling gait, lordosis
-Gower’s maneuver*: pt uses hands to get up from
floor
-Develop limb flexion contractures and scoliosis
-Males affected
-Ambulation preserved until age 15, many children
remain ambulatory into adulthood
-S/s begin during adolescence or young adulthood
-Myotonia (delayed relaxation after muscle
contraction), weakness and wasting of distal limb
muscles (esp hands) and facial muscles (ptosis),
cardiomyopathy
-Onset: early childhood to adulthood
-Weakness in proximal limbs (shoulder girdle),
severe loss of function by age 20
-Begins age 7-20, normal life expectancy
-Slow progression, *difficulty whistling and raising
the arms
-Infantile: facial, shoulder, hip girdle weakness,
rapidly progressive: scapula winging on PE
-Acute: Risks: emotional stress, caffeine, pain,
change in sleep schedule
-Chronic (3 nights/wk for 1 mo): depression, stress,
pain, medical conditions, chronic fatigue syndrome
-Presents as difficulty falling asleep, wakefulness
during night, early morning awakening
-Obstructive: intermittent obstruction of upper
airway by the tongue  asphyxia
-Central: respiratory center in the brain
-Presents as male 3-60 with h/o snoring and
excessing daytime sleepiness, nocturnal gasping,
-MC form of muscular dystrophy
in whites and adults
-Affects both M and F
-2nd MC muscular dystrophy in
US
-M = F
-Affect multiple genes
Fascioscapulohumeral Dystrophy
-Autosomal dominant disorder (D4Z4):
weakness of facial muscles and
shoulder girdle
-3rd MCC of muscular dystrophy
in US
Insomnia
-Loss of restorative sleep, despite
opportunity
-Difficulty falling asleep, staying asleep,
waking up too early
-More common in women,
increased age, psych problems
and comorbidities
Sleep Apnea
-Periods of apnea/breathing cessation
during sleep
-Cessation of airflow through nose and
mouth for 10 sec or longer
-Risk: males, increasing age and
obesity, large neck girth
-Suspicion with
characteristic clinical
findings, age @ onset, fhx
-EMG: rapidly recruited,
short duration, low
amplitude motor potentials
(weak, insufficient
contractions)
-Muscle bx: necrosis,
marked variation
-*Elevated CK levels
-*Immunostaining analysis
of dystrophin
-*DNA mutation analysis
(PCR, southern blot)
hip dislocations, scoliosis, spastcty
-Geneticist
-Neurosurgeon: identify & tx
hydrocephalus, tethered spinal cord,
spasticity; rhizotomy, reconstructive
surgery
-Gastroenterologist, nutritionist
-Pulmonologist
-Multidisciplinary learning disability
team
-Supportive: moderate exercise,
passive exercises (low intensity, nonweight bearing), ankle-foot orthoses,
leg braces, weight control
-Genetic counseling
-Daily prednisone – protects muscle
mass and function
-Corrective surgery
-Clinical, age @ onset, fhx
-*DNA testing TOC
-Ankle foot braces, drug therapy for
myotonia (mexiletine)
-Muscle histology,
immunocytochemistry,
Western blot, genetic test
-Confirmed by DNA testing
-Prevention of contractures: exercise,
braces, surgery as needed
-Sleep diary ~1-2 weeks
-Polysomnography:
electrooculogram, EMG,
ECG, breathing mov’t, pulse
Ox
-R/O other disorders
-Polysomnography:
overnight sleep studies –
measure ventilation, arterial
O2, heart rate
-Short term: Benzos (Flurazepam),
Halcion or non-benzo hypnotics
(ambient, sonata) – limit to < 10 days
-Antihistamines: can lead to morning
drowsiness
-Sleep hygiene
-Mild to moderate: weight loss, avoid
aggravating factors, sleep in lateral
position
-Severe: Nasal CPAP (continuous
positive airway pressure) – provides
-Physical therapy, braces, albuterol:
increases muscle mass and strength
moderate obesity and large neck circumference,
HTN, behavioral disturbance
-Cardiorespiratory: increased L ventricular afterload,
bradycardia, tachycardia, HTN
-Muscle weakness w/o loss of consciousness
(cataplexy), daytime sleep of 30 min or less,
hallucinations, both visual and auditory, sleep
paralysis
Narcolepsy
-Daytime sleep attacks – abrupt
transition to REM sleep
positive pressure preventing occlusion
of airway
-Uvulopalatopharyngoplasty
-Hx, R/O other diagnoses
(seizures), sleep studies
-Stimulant meds: Methylphenidate
(Ritalin), wake promoting therapeutics
-REM sleep depression with
antidepressants, adequate nocturnal
sleep time, planned daytime naps
Circadian Rhythm Disorders
-Non-24 hr sleep-wake syndrome
-Time zone change: Jet lag
-Shift work sleep disorder
-Change in sleep phase disorder
Periodic Movement Disorder
-Common in blind pts
Restless Leg Syndrome
-Centrally acting dopamine receptor
antagonists reactivt sx
-Common in middle age
-Female predilection
Parasomnias
-Movements and behaviors not
expected that occur during sleep or are
exaggerated by sleep
Myasthenia Gravis
-Acquired autoimmune disorder with
weakness of skeletal muscles
(progressive through the day) and
fatiguability on exertion
-Autoantibodies to AChR @ NMJ of
skeletal muscles or antibodies against
muscle-specific kinase (MuSK)
-Associated with other auto-immune
disorders: Hashimoto’s, SLE, RA
Lambert-Eaton Myasthenic
Syndrome
CLASSIFICATION:
I: Ocular muscle weakness,
weakness of eye closure, all
other muscle strength normal
II: Mild weakness affecting
other than ocular muscles,
ocular muscle weakness of any
severity
III: Moderate weakness
affecting other than ocular
muscles, ocular muscle
weakness of any severity
IV: Severe weakness affecting
other than ocular muscles,
ocular muscle weakness of any
severity
V: Intubation, with or w/o
mechanical ventilation
-Internal sleep/wake rhythm and external differ
-Difficulty falling asleep/awakening, poor sleep habit
-Episodes of repetitive mov’t of large toe with flexion
of ankle, knee, hip during sleep – Stage I/II
-Compulsion to move legs with or w/o paresthesias,
worse @ rest, night, improves with activity
-Motor restlessness: pacing, tossing/turning, rubbing
their legs
-Present at evening/night
-May also have sleep disturbance, daytime fatigue,
normal neuro, leg mov’ts during sleep
-Sleep terror: abrupt, terrifying arousal from sleep;
NREM stage III/IV; fear, sweating, tachycardia,
confusion, screaming
-Nightmares: occur during REM sleep
-Teeth grinding, bed wetting
-Sleepwalking – child ages 6-12, stage III/IV, NREM
-Fluctuating weakness increased by exertion:
pathognomic
-Weakness increases during day, improves with rest
-Sensory exam, DTR normal
-MC initial sx: EOM weakness, ptosis, diplopia,
blurred vision (mimics MS) – EOM weakness
asymmetric
-Facial muscle weakness: bilateral- mask like face
-Bulbar muscle weakness: nasal voice, nasal
regurgitation of foods and liquids, jaw hangs open,
slurred speech, difficulty chewing/swallowing 
aspiration, *weakness of neck muscles: neck flexors
-Limb muscle weakness: proximal≫distal, UE>LE
-Respiratory muscle weakness (*myasthenic crisis):
immediate intubation, weakness of intercostals
muscles and diaphragm leads to resp failure
-Progress from mild to severe dz from wks→ mos
-Exacerbation with: Abx (AMG, FQ), BB, cardiac
medications, anticholinergics, *premenstrual,
pregnancy, post-partum
-Proximal muscle weakness and hyporeflexia
-Sx improve with repeated stimulation**
-Artificial light prior to wakening
-R/O iron deficiency, check
ferritin
-Dopaminergic agent: Sinemet,
pergolide, bromocriptine
-Benzodiazepenes
-Reassurance, pt education
-Eliminate secondary
etiologies
-Benzos (suppress stage III/IV)
-TCAs
-Anti-acetylcholine receptor
antibody (most specific):
false positive with
thymoma, Lambert-Eaton,
SCLC
-Anti-MuSK antibody
-Thyroid function test
CXR
-*Chest CT to identify
thymoma
-MRI of brain
-Repetitive Nerve
Stimulation – MC (rapid)
-Single-fiber
electromyography – most
specific
-**Pharmacological testing
(edrophonium, Tensilon
test)- marked improvement
of sx
-AChE inhibitors (neostigmine,
pyridostigmine)
-Thymectomy- first-line, leads to
symptomatic relief and potential
remission
-Corticosteroids: for pts responding
poorly to AChE inhibitors and have
already undergone thymectomy
-Azathioprine, cyclosporine,
mycophenolate mofetil
-Plasmapheresis or IV Ig to remove
antibodies
-Abnormality of Ach release @ NMJ:
auto-antibodies to presynaptic Cachannels
Dyskinesia
-Involuntary, non-repetitive,
occasionally stereotypical mov’ts
affecting distal, proximal, axial
musculature in varying combinations –
Basal ganglia disorders
Tremor
-Rhythmic, alternating involuntary mov’t
caused by repetitive muscle contraction
and relaxation (oscillation)
-Associated with SCLC
-MC movement disorders
-Associated w/age and
neurological disease
Chorea
-Brief, rapid, jerky, purposeless,
irregular involuntary mov’t of distal
extremities and face, may merge into
purposeful or semi-purposeful acts that
mask involuntary motion → →→
Athetosis
-Writhing mov’t, often with alternating
postures of proximal limbs that blend
into flowing stream of mov’t
Dystonia
-Sustained abnormal postures and
muscle contractures, disruptions of
ongoing mov’t resulting from alterations
in muscle tone
Myoclonus
-Rapid, brief irregular contraction of a
muscle or group of muscles
Tic
-Brief, rapid, simple or complex
involuntary mov’ts that are stereotypical
and repetitive
LEADS TO∷
Tourette’s Syndrome
-Multiple complex motor and vocal tics,
coprolalia
-Not mediated through normal motor
pathways, truly involuntary and
suspected to occur in response to
external cue
-Male predilection
-Frequency and severity
symptoms improve into
adulthood
-Associated with OCD, ADD
-Difficulty performing voluntary movements
-Extrapyramidal
-Hyperkinetic: excessive amt of spontaneous motor
activity, abnormal involuntary mov’t
-Hypokinetic: purposeful motor activity absent
(akinesia), or reduced/slowed (bradykinesia)
-Essential tremor: Slow tremor that affects hand,
head, voice – may be unilateral, minimal at rest,
increases with age and movement, enhanced by
anxiety, stress, fatigue, metabolic/drug changes
-Postural tremor: maximal when body part is
maintained against gravity, lessened by rest
-Rest: Maximal @ rest, less prominent with activity
-Intention (Kinetic) tremor: maximal during
voluntary or purposeful mov’t toward a target:
cerebellar damage
-Chorea and athetosis often occur together
(choreoathetosis)
-Manifestations of basal ganglia disease: Wilson’s,
Huntington’s*
-Acute onset usually due to toxins (levodopa,
dopoamine agonists), pregnancy (chorea
gravidarum), hyperthyroidism and association with
Rhematic fever (Sydenham’s chorea)
-Essential tremor improves with
alcohol, propranolol, benzos
-Examples: cervical dystonia, spasmodic tortecolus
-Frequently causes twisting, abnormal posture
-Anticholinergics, botulinum toxin
injection (weakens involuntary
contractions)
-May occur normally as a person falls asleep
-Abnormal myoclonus: metabolic derangements
(uremia), degenerative (Alzheimer’s), after severe
closed head trauma or hypoxic-ischemic brain injury
-Simple: (blinking, nose twitch, eye roll, shoulder
shrug, jaw or head jerks), usually occur in
childhood, often occur repetitively in one location
-Complex: coordinated, sequential, resemble
fragments of normal behavior: clapping, drumming
fingers, picking scabs, kissing, touching, hitting self
-Simple tics usually presenting symptom (MC facial
tics, 2nd MC neck/shoulder tic)
-Presenting vocal tics usually noises – utterance of
words become pathognomic
-Tics wax and wane, change character, most pts
have 1-2 tics that endure
-Premonitory feeling precede motor and vocal tics
-Avoid social encounter: provoked by caffeine,
-Correction of underlying metabolic
abnormalities, Benzo’s anticonvulsants
-Neuroleptics (haloperidol): block
postsynaptic dopamine receptors,
benzos
hormones, excitement
-Lessened by relaxation, may be suppressed
voluntarily for a time
-Reduction in tics when distracted
-Most severe: self-injurious tics, coprolalia,
copropraxia, animal sounds, echolalia, talking with
different accents
-High incidence of learning disabilities
-Usually affects arms more than legs: severe form of
chorea
-Biballism: occasional bilateral mov’ts occur
-Caused by lesion, usually infarct, in contralateral
subthalamic nucleus
Hemiballismus
-Violent, continuous proximal limb
flinging mov’ts confined to one side of
the body
Drug-Induced Mov’t Disorders
Acute Dystonia
Akathisia
Parkinsonian-like symptoms
Tardive Dyskinesia
-MCC by drugs that block
dopamine receptors
(neuroleptics)
-Days to weeks after neuroleptic
-Occurs weeks to months after
therapy
-Follows >6 mo (often many
years) of therapy
*Neuroleptic Malignant Syndrome
Huntington’s Disease
-Incurable, adult-onset autosomal
dominant inherited dz with: dementia,
behavioral change, involuntary mov’ts
-Gradual onset and slow progression
leading to disability and death
-Abnormality of chromosome 4 that
causes toxic accumulation of protein
huntingtin which accumulates in clumps
within brain cells – death of GABA →
increases dopamine
-Symptoms don’t develop until
after 30 years of age
-By the time of diagnosis, the pt
has usually passed on the gene
-Mean age of death 51-57,
duration of illness approx 20
years
Parkinson’s Disease
-Adult onset gradually progressive
neurodegenerative disorder of the
extrapyramidal system
-Loss of dopaminergic neurons in
substantia nigra, presence of Lewy
bodies* (concentric, eosinophilic,
cytoplasmic inclusions)
-MC neurological disorders
-Male predilection
-Usually self-limiting (6-8 weeks)
-Treat with dopamine depleting agents
-Sustained muscle spasms of face, neck, trunk
-Subjective sensation of motor restlessness
-*Resting tremor, bradykinesia, rigidity, postural
instability
-Involuntary facial and tongue mov’t, rhythmic trunk
mov’t, choreoathetoid mov’t of extremities
-Muscular rigidity*, fever, tremor, AMS, autonomic
instability – blocks dopamine receptors
-Mov’t, cognitive and behavioral disorder: chorea MC
-Athetosis → dystonia → Parkinsonian features →
akinetic-rigid syndrome, spasticity, clonus
-Dysarthria, dysphagia common – abnormal eye
movements early in disease
-DTRs variable
-Dementia and psychiatric features earliest
indicators: irritable, unkempt, loss of interest, slow
cognition, impaired intellect, memory changes,
decreased verbal fluency, attention deficit, executive
function, abstract thought
-Behavioral: depression, mania, increased rate of
suicide, anxiety, OCD, sexual and sleep disorders,
personality changes, psychosis, impulsiveness,
hostility, agitation, hallucinations, paranoia
-Unilateral pill-rolling resting tremor
-Initial: fatigue, depression, constipation, sleep
disturbance
-Decreased dexterity, lack of coordination, gait
disturbances (affected arm doesn’t swing, feet drag,
festinating gait), stooped posture, poor balance,
problems getting up from chair, neglect of
swallowing, drooling, sexual dysfunction, seborrheic
dermatitis, blepharitis, bradykinesia (small
handwriting, low volume speech), lead pipe rigidity
(cogwheel rigidity)
-Withdraw drug, anticholinergics, anti
histamines
-Presymptomatic detection
of dz ~ genetic linkage
analysis
-CT or MRI: cerebral
atrophy, ventricular
enlargement, atrophy of
basal ganglia: measure
bicaduate diameter*
-History
-CT/MRI for evaluation of
dementia
-Remove drug, anticholinergics
(amantadine, diphenhydramine)
-Gradual reduction of offending drug
-Cessation of drug, ICU, hydration and
cardiopulmonary function – resolves on
its own over weeks, dantrolene to
reduce muscle contractility
-Genetic counseling, speech, physical
and occupational therapy, psychiatric
referral
-Symptomatic: choreoathetosis: benzos
(clonazepam, reserpine, tetrabenazine)
-Depression: SSRIs, ECT
-Bradykinesia and rigidity: levodopa,
dopamine agonists
-Antipsychotic medications and mood
stabilizers
-Neurodegenerative dz treated long
term
-L-Dopa + Carbidopa (Sinemet) DOC
-Dopaminergic agonists: bromocriptine,
pergolide, pramipexole
-Anticholinergics: amantadine,
trihexyphenidyl, benztropine
-MAO-B inhibitor or COMT inhibitor
-Neuro consult