AD

Sx- late loss of early memories

Tx- ACHEi donepezil, tacrine, rivastigmine, galatamine; donepezil preferred bc least
GI effects
Memantine is neuroprotective in pts with moderate to severe dementia
Tacrine has severe hepatic toxicity

Child abuse (seizure): somnolent, vomiting, seizure (increased ICP)

-next step- perform retinoscopic exam
- CT- cerebral edema, bilateral subdural hemorrhages (patho for child abuse)
EXAM BEFORE LABS
Vs. Reye sx (3 wks not 3 mos before sxs)

Cluster HA
sx: autonomic sx (conjunctival injection, lacrimation, rhinorrhea, nasal congestion,
ptosis)
Tx: O2, sumatriptan, octreotide, lidocaine, ergots; avoid alcohol during attacks
(trigger)
Ppx: glucocorticoids during verapamil 2wk bridge

Trigeminal neuralgia
Tx- carbamazepine or phenytoin; gabapentin, lamotrigeine, amitriptyline
NO ASPIRIN OR NSAIDS

Brainstem glioma
Sx: child, neck stiffness or discomfort before torticollis; long history of isolated
cranial nerve deficit or contralateral hemiparesis; medullary tumors- dysphagia,
nasal speech, apnea; cerebellar deficits- nystagmus, ataxia, nausea, vomiting
Dx: MRI

Medulloblastomas: cerebellar vermis and grow down into forth ventricle

-neuroectoderm precursors; sheets of undifferentiated cells with scanty cytoplasm
and immunohistochemical features of neuronal or astrocytic differentiation;
extention to the cerebellar surface produces sugar coating or drop metastasis to
spinal cord through CSF is characteristic spread
Sc: increased Icp (nocturnal or morning headaches, nausea, vomiting, AMS); truncal
ataxia, head tibulation, nystagmus, unsteady gait
- highly responsive to radiation and chemotherapy

Ependymoma: lateral or fourth ventricle; mass filling the fourth ventricle

- tend to recur after surgical resecFtion

Hemangioblastoma: cerebellar hemispheres; cyst with mural contrast-enhancing

nodule
a/w von Hippel-Lindau syndrome

Meningioma: MC benign intracranial tumor; dural-attached mass; poor prognosis

because of repeated recurrence after surgery and inevitable progression to high-
grade tumor
Pilocytic astrocytoma: well-circumscribed astrocytoma of children in cerebellum
(hemisphere) and diencephalic region

Oligodendroglioma: rarely in children, 5% of all brain tumors

Benign paroxysmal torticollis
Sx: first few months of life, benign self-limited, recurrent episodes of head tilt, often
accompanied by vomiting, pallor, irritability, ataxia, drowsiness; episodes last few
hours sometimes days, occurring less often as child gets older and disappear by age
5

Dystonic reaction: phenothiazines, carbamazepine, phenytoin

Sx: fixed upper gaze, neck twisting grimace, clenched jaw, difficulty of speech; very
painful

MM: plasma cell proliferation; neuro sxs 2/2 hyperCa, peripheral nerve amyloidosis,
nerve ischemia from hyperviscosity, spinal cord compression from myeloma tumors
or vertebral fractures
Sx: cord compression from tumor is emergency because can prevent complications
(pain worsened by lying down or Valsalva-like activities/coughing sneezing,
weakness, numbness, of lower extremities
- peripheral nerve amyloid infiltration usually manifests as carpal tunnel syndrome
- steroid-induced avascular necrosis of femoral head manifest as pain and
decreased range of motion
- thalidomide- neurotoxicity manifests as asymmetric, painful, peripheral
paresthesias, sensory loss, muscle cramps, weakness. Thalidomide phocomelia
occurs in offspring of women resulting in abnormal shortening and malformation of
extremities

Meniers: hearing loss, vertigo, nausea, vomiting, 20 min to 24 hrs

Tx- meclizine, scopolamine
Vs. acoustic neuroma: vertigo, tinnitus, sensory hearing loss (MRI)
Vs. MS: nystagmus
Vs. benign positional vertigo: no hearing loss; dx- hallpike maneurver
Vs. benign recurrent vertigo=vestibular neuronitis- no change in hearing

Arnold-Chiari malformations: small posterior fossa

1 (MC)- usually asx, downward displacement of cerebellar tonsils through foramen
magnum; late presentation with headache, neck pain, urinary frequency, and
progressive lower-extremity spasticity
2 - downward displacement of cerebellar vermis and medulla through foramen
magnum, leading to obstruction of CSF flow and hydrocephalus. Associated with
lumbar myelomeningocele and syringomyelia
Fourth ventricle is elongated and brain stem is kinked
Dandy-walker: large posterior fossa, absent cerebellar vermis, large
ependymal-lined cyst that represents expanded 4th ventricle (roof of 4th ventricle
fails to form resulting in a cystic expansion of the 4 th ventricle into the posterior
fossa)
Holoprosencephaly- incomplete separation of cerebral hemisphere along midline
leading to a single ventricular cavity enclosed within forebrain
Ancencphly f- neural tube defect

Myelomeningocele:
Sx- flaccid paralysis with no reflexes of lower extremities, loss of touch and pain
Associated with clubffot or congenital hip dysplasia
Seizure
- dont put anything gin the mouth
- dont resrain patient
- do place patient on the side
- do place pillow under head
- do loosen itght clothin aound the neck
- do remove sharp objects from surroundings
- do call ambulance only if >5-10min

Absence
Dx: EEG with 3Hz spikes and slow waves
Tx: ethasuximide, valproate, lamotrigine

Dementia
Lewy body dementia- hallucinations with movement problems (jerking with
resistance), fluctuating course, with decrease in visuo-spatial skills, and verbal
fluency
Picks disease- disinhibition and personality changes, visuospatial deficits
CJD- myoclonus

Botox- preformed toxin inhibits Ach release

Sx: symmetric descending flaccid paralysis, bulbar palsies, normal sensation; ptosis,
blurred vision diplopia, dysarthria, dysphonia, dysphagia
ALS- pure motor, GBS- pure sensory, organophosphate- fasciculation, excessive
bodily secretions

HIV encephalopathy
Sx: psychomotor retardation, depression, and dementia (with symmetric findings on
imaging)

Spinal cord transection

Sx: quadriplegia, respiratory insufficiency, anesthesia below affected level,
neurologic shock, loss of rectal and bladder sphincter tone, urinary and bower
retention
Syringomyelia
Communicating- a/w Arnold-Chiari malformation, noncommunicating- a/w spinal
cord injury
Subacute combined degeneration of the spinal cord: Vit B12 deficiency (distal
sensory and UMN deficit), distal paresthesias and weakness of extremities, followed
by spastic paresis and ataxia
- Combined deficit of vibration and proprioception with pyramidal sings
(plantar extension and hyperreflexia)
Syringobulbia: syrinx at medullahypoglossal deficit and trigeminal impairment in
pain and temperature
Sx: dysphagia, nystagmus, pharyngeal and palatal weakness, asymmetric weakness
and atrophy of the tongue, and sensory loss involving primary pain and temperature
senses in distribution of Trigeminal nerve

ADEM: hyperintense lesions in white matter of brain and spine; after viral
vaccination (esp. rabies vaccine), viral illness, or atypical infection; very acute
Vs. ALS: more insidious
Vs. GBS: peripheral demylination not CNS
Vs. polymyositis: muscular defect, not neuronal

Endocarditis->epidural abscess->spinal cord compression (bowel incontinence)

- staph a., gram-neg rods, strep, coag-neg staph
Epidural abscess:
dx- MRI (shows early inflammatory changes)- indications for imaging: <18 >50,
infection risk like IVDA, immunosuppression, oseoporosis
GBS: paresthesias and ASCENDING WEAKNESS
Vs. spinal cord compression (nonprogressive/nonascending)
Dx: best initial- CSF culture; best confirmatory- nerve conduction studies (takes 2-3
weeks for clinical findings)
Miller-Fisher variant- starts with cranial-nerve deficits (uncommon)

Myasthenia Gravis
Dx: EMG with repetitive stimulation
- Tensilon/edrophonium can be positive with ALS

Complex regional pain syndrome (reflex sympathetic dystrophy): unilateral upper

extremit after trauma, resulting from aberrant nerve healing
1. weeks to months: severe pain from innocuous stimuli in addition to redness,
swelling, sweating, and joint pain
2. similar pain with atrophy of skin and its appendages, coolness and pallor
3. continued atrophy of skin and underlying muscles and flexion contracture of the
affected extremity, resulting in claw-hand deformity

Dupuytren contracture: palmar fibromatosis; fibrosis of palmar fascia, usuallyh

bilaterally, resulting in flexion deformity of the fingers; associated with alcohol use,
diabetes, and family hisotyr
Erb-Duchenne palsy: forceful stretching between head and shoulder; damage to
upper trunk of brachial plexis
Sx: arm is pronated and medially rotated waiters tip
Kulmpke palsy: sudden upward pulling of the arm resulting in damage to the lower
trunk of brachial plexus; median and ulnar nerves are primarily damaged leading to
paresis of intrinsic muscles of the hand

Essential benign tremor: head bobbing arms legs face tremor

Tx: propranolol, primidone, alcohol

ALS:
Dx- EMG shows widespread axonal death
Pgx- death within 3-5 years after diagnosis due to respiratory muscle failure
Migraine: aura (scotoma, fortification/zigzag, scintilla/flashing lights, unilateral
paresthesia or weakness, hallucintions, or hemianopsia
-worsen with Valsalva maneuvers
PPx: propranolol, timolol, valproate, amitriptyline; 2-3 mos trial
Abort: sumatriptans, ergotamine +/- caffeine, NSAIDS

Ring-enhancing lesions (MAGIC DR. L)

- neurocysticercosis: gray-white junction, MCC of seizures in developing world
- toxoplasmosis
- lymphoma
-abscess
-tuberculoma
- glioblastoma, metastasis
- demyelination (incomplete ring)
-infarct/infection
-radiation necrosis or resolving hematoma

Lamber-Eaton myastheniac syndrome is associated with lung cancer: next step is CT

chest

Otosclerosis: a/w late teens and early twenties; more rapidly progressive with
pregnancy or OCP use
Tx- hearing aid (conductive hearing loss), removal of stapes

GBS: a/w HIV and campylobacter

CSF leak:
Dx: beta-2-transferrin (best), glucose oxidase paper (fast but very unreliable)

Spinal stenosis
Sx- Spurling sign (radicular pain with neck extension and lateral flexion),), reduced
ROM of neck
- pseudoclaudication
Dx- Xray (atraumatic neck pain in >50yo)
Lhermitte sign (electric shock with neck flexion)- multiple sclerosis, cervical disc
herniation
CSF leak
Comp: meningitis (most common and most severe) 2/2 strep pneumo infection of
exposed CSF
Risk of meningitis from atraumatic causes is much higher than in traumatic CSF
rhinorrhea (40%>10%)
Strep pneumo>H flu

Subarachnoid hemorrhage (vs. Cluster headache): constant with focal neurological

deficits

Lung cancer: constitutional symptoms + cough

Lymphoma: constitutional symptoms
Teratoma: calcifications and heterogenous densities
Mediastinal mass: lymphadenopathy,teratoma, lymphoma, enlarged thyroid,
thymoma

Malignant astrocytoma: MC primary malignant neoplasm

MS tend to have hyporeflexic bladder->overflow incontinence->Tx: bethanechol

Imipramine: mixed a-agonist and anticholinergic-> treats hyper-reflexic bladder
optic neuritis- painful eye movements, blurry vision; flame-shaped hemorrhages,
optic atrophy
Tx- corticosteroids

Metastatic cancer of spine (breast, lung, prostate) suggestive of epidural

compression of spinal cord
Sx- sudden incontinence with weakness and chronic back pain
Next best step: IV dexamethasone, then MRI, then surgery
Complications: rapid paraplegia

NPH
Dx: CT w/o contrast or MRI head then LP
Contast is for tumors or infectious lesions

Cavernous sinus thrombosis: fever, retro-orbital headache, swollen protruding eye;

CN4 and 6 palsy

Mononeuritis multiplex: painful asymmetric asynchronous sensory and motor

peripheral neuropathy, isolated damage to >1 nerve
Most commonly seen with systemic vasculitis (ex. Churgg Strauss syndrome, SLE,
and cryoglobulinemia)
Cryoglobulinemia: palpable purpura, arthalgias, peripheral neuropathy,
hypocomplementemia

ACA: contralateral weakness and cortical sensory loss in the contralateral leg with
or without weakness in the arm; urinary incontinence, confusion, and behavioral
disturbances are likely
MCA: contralateral hemiplegia, hemisensory loss, aphasias, and homonoymous
hemianopia
PCA: contralateral homonoymous hemianopia, visual hallucinations, agnosias, alexia
without agraphia, spontaneous pain, CN3 palsy paresis of vertical eye movement

Vertebral artery (medial medullary infarct)

Vertebrobasilar ischemia (VBI): dizziness and vertigo +/- dip
Lateral medullary syndrome (Wallenberg; PICA)- ipsilateral paralysis of vocal
cord, dysphagia, palate droop (dont pick a hoarse that cant eat)
Medial medullary syndrome (vertebral artery) tongue deviates to side of
lesion
Locked-in syndrome (basilar artery) paralysis of all extremities, heavy
sensation deficits, dysphagia, difficulty in respiration
Subclavian steal syndrome

Encephalitis: headache and AMS

GBS: symmetric ascending paresthesias and weakness
Dx- Nerve conduction study (demyelinating neuropathy), CSF culture for
albuminocytoogic dissociation

Lumbar disk herniation:

Sx- several months of vague, aching pain before sudden onset of neurogenic pain
precipitated by a
forced movement (extremely severe like an electric shock shooting down the leg
exacerbated by
coughing, sneezing, or defecating. Unable to ambulate and holds affected leg in
flexed position)

Spinal stenosis:
Sx-pain worsened by extension and improves with flexion of hips, knees, or lumbar
spine
-easy going uphill but pain (stooped over) while going downhill (upright)
Metastatic disease
-progressive back pain worse at night and unrelieved with rest or positional changes
Vertebral compression fracture
- from minor trauma or minor fall, resulting in acute back pain after trauma

ASA infarct
Sx: flaccid paralysis, incontinence, loss of pain and temperature

Acute dystonia (torticollis)

Tx- DA-agonist (L-DOPA/carbidopa), anticholinergic/antihistaminic
(diphenhydramine, benztropine,
trihexyphenidyl), muscle relaxant (benzodiazepine), if focal botox injection

Pseudotumor cerebri (idiopathic intracranial HTN)

Ex- women of childbearing age, OCP use, tetracycline use
Sx- headache and visual disturbances worse with coughing or sneezing
Dx- MRI- flattening of posterior sclera, empty sella, or distension of perioptic
subarachnoid space
Tx- acetazolamide, LP

Amputation
Viable- no deficits with audible pulses
Marginally threatened: minimal sensory loss in toes, no weakness, inaudible arterial
pulses, audible venous pulses; salvageable if treated fast
Immediately threatened: greater sensory loss further up than toes; resting pain,
mild to moderate weakness, no audible arterial pulses, audible venous pulses;
requires immediate revascularization
Irreversible damage: amputation to prevent thrombus or embolus

Presumed Toxo unresponsive to treatment is likely B-cell lymphoma

Next step: brain biopsy
Sx- fever, night sweats, weight loss, fatigue, and s/s of mass occupying lesion
(confusion, focal weakness, hemiparesis, headache)
Wernicke:
1. encephalopathy- disorientation and indifference
2. oculomotor dysfunction- nystagmus, lateral rectus palsy, and conjugate gaze
palsy
3. gait ataxia
Tx- thiamine + glucose (+ magnesium)
Korsakoff amnesic syndrome: anterograde and retrograde amnesia; irreversible

Cerebellar hemorrhage/hematoma: abrupt onset; inability to walk due to imbalance,

vomiting, neck stiffness, headache, gaze palsy, and facial weakness

Anticoag after hemorrhage

Breakthrough seizure
Next step: drug level

Pseudotumor cerebri: signs of elevated ICP (headache, dimished vision,

papilledema) with normal MRI
ICP>200; LP needed for diagnosis
Criteria- awake and alert, s/s of increased ICP, no focal findings except CN6 palsy,
normal CSF except increased ICP, normal imaging findings, no other causes of
increased ICP identified
Common causes- vitamin A, OCP, steroids, trauma
Tx- acetazolamide (inhibits choroid plexus CA to decrease CSF production) preferred
over serial LPs
Steroids if continued visual problems despite medication

Fat embolism:
Petechiae in setting of long bone trauma is patho
Sx- dyspnea, tachypnea, AMS
Dx- multifocal petechiae in white matter

Subarachnoid hemorrhage
Sx- headache warning leak before signs of meningismus
CT, if negative do LP for xanthochromia; CT is less sensitive the longer you wait

Tuberous sclerosis
Tubers in brain (collections of various types of neural tissue), often calcify and
detectable by CT; neurologic impairment is proportional to the number of tubers
present
Ashleaf spots
Infantile spasms (brief symmetric contractions of neck, trunk, and extremities),
often confused as colic pain and GERD
EEG shows hypsarrhythia- high-voltage, asynchronous, chaotic slow-wave
activity
Tx: ACTH to decrease CRH that causes excess excitability

Infant with generalized hypotonia, no deep tendon reflexes, and fasciculations of

the tongue
Type I spinal motor atrophy (Werdnig-Hoffman disease)- progressive denervation of
muscle and subsequent muscle fiber atrophy
-motor nerve conduction is okay (compared to peripheral neuropathy)
Dx- survivor motor neuron (SMN) gene in the blood, muscle biopsy showing
perineural denervation

Enthesopathy- inflammation with associated pain and tenderness at insertion sites

of tendons and ligaments onto bone
Associated with seronegative spondyloarthropathies (ankylosing spondylitis and
reactive arthritis)
- symptoms usually improve with activity vs. spinal stenosis

Brain metastases
Tx- whole-brain radiation (rather than chemoless effective) to improve neurologic
deficits caused by the metastases and edema, and to prevent deterioration of
neurological function
Surgical resection single metastasis

Juvenile Nasopharyngeal angiofibroma- markedly vascular and causes epistaxis

Sx- epistaxis, nasal discharge, rhinorrhea, nasal obstruction recurrent otitis media,
conducive hearing loss, diplopia, eye pain, rarely anosmia
Dx- grayish-red mass in posterior nasal pharynx; confim with MRI or CT with
contrast

Inverted papilloma- inversion of epithelium into the connective tissue stroma

Dx- red, polypoid masses attached to the lateral nasal wall, middle turbinate or
septum

Pyogenic granuloma- tumor associated with capillary proliferation, history of trauma

Rhabdomyosarcoma- epistaxis, middle ear effusion, face pain, damage to cranial
nerves

Polyneuropathy- lower motor neuron lesions

Polymyositis- nomuscle fasciculation

Any acute spinal cord injury

1st: high dose IV steroids after neuro exam (decrease pressure on spinal column)
within 8 hrs of injury
Brown-Sequard: ipsilateral loss of everything, contralateral loss of pain and temp

If need anticoag but has bleed, use IVC filter

PML
Dx- white matter lesions that are patchy, focal, and nonenhancing; oligodendorcytes
have intranuclear inlusions
Sx- hemiparesis, visual deficits, ataxia, aphasia, decreased cognition and coma in
advanced disease

Internal carotid artery stenosis

- risk for watershed infarcts
HIV encephatlopathy
Dx- confluent and symmetric appearance on imaging
Sx- psychomotor retardation, depression, and dementia

Primary lymphoma of brain

Ex- hx of EBV
Peds:
Neonatal seizures:
Ex- MCC is hypoxic-ischemic encephalopathy (HIE)
Tx- ABCs, admit to NICU for monitoring, benzo for status epilepticus
Other causes- infection, electrolyte aabnormalities (hypoglycemia), structural,
organic acidemias, aminoacidopathies, urea cycle defects

Intraventricular hemorrhage
Ex- preterm infants; choroid plexus is not fully developed but still highly
vascularized; blood fills the lateral ventricles and could obstruct the flow of CSF
producing hydrocephalus
Damage is from hydrocephalus damage surrounding the whie matter
(periventricular white matter), but mostly from hypoxic-ischemic episodes where
the periventricular white matter undergoes liquefaction necrosis (periventricular
leukomalacia/periventricular white-matter necrosis)
Sx- Cerebral palsy- spastic diplegia or spastic quadriplegia

Athetoid Cerebral palsy- dyskinesia from damage to basal ganglia (2/2 kernicterus,
asphyxia, or genetic/metabolic diseases)
Sx- infants are hypotonic and becomes rigid with dystonic movements over the
years
Craniopharyngiomas
Dx- heavy calcifications and viscous yellow fluid content; micro tumor composed of
islands of keratinizing epithelium with multifocal calcific deposits similar to tooth
related tumors (ameloblastoma)

Ganglioma:
Dx- mixture of neurons and glial cells most commonly in the temporal lobe
Sx- seizures

Von Hippel Lindau: chrm 3

Hemangioblastoma (cyst-mural nodule), brain neoplasms, retinal angiomas, clear
cell renal cell carcinoma, pheochromocytomas
Cyst-mural nodule- hemangioblastoma, pilocytic astromcytoma in
cerebellum, ganglioma in cerebral hemisphere, and craniopharyngioma in
suprasellar region
Li-Fraumeni- p53 tumor suppressor gene mutation
Array of malignant neoplasms (breast cancer, brain tumors/gliomas, skin cancer)

NF1- Schwann cell tumors of peripheral nerves, caf-au-lait

NF2- multiple brain tumors (CN8 tumor), meningioma, and gliomas

Tuberous sclerosis- hamartomatous malformations of organs

Huntingtons disease
Tx- antidopaminergics to block involuntary movements (haloperidol, olanzapine,
risperidone, aripiprazole)
Dopamine agonists (bromocriptine) can worsen movements

CSF leak/low CSF volume headache:

-worsens with being upright, improves with laying supine
1. Confirm via beta-2-transferrin
2. Image head CT

MS: optic neuritis with normal funduscopic exam, then papillitis (disc optic disc
swelling)
Tx- IV methylprednisolone; long term is IFN-beta

Focal dystonia (writing cramp)

- Hyperextension or hyperflexion of the wrist and fingers, triggered by repetive
motions

Cauda equine syndrome- LMN findings ONLY

- Gradual and unilateral
- More severe radicular pain
- Less low back pain
- Strength: asymmetric areflexic paraplegia, with atrophy, sphincter
dysfunction presents late
- Reflexes: both knee and ankle jerks affected
- Impotence less frequent
Conus medullaris syndrome- UMN findings and LMN findings
- Sudden and bilateral
- Less severe radicular pain
- More low back pain
- Strength: symmetric, fasciculation
- Relfexes: ankle jerks affected
- Sphincter dysfunction early
- Impotence frequent

Postherpetic neuralgia (AFTER acute episode of zoster): postinfectious inflammation

and nerve damage result in persistent pain after resolution of herpes zoster
Tx- desipramine, gabapentin, pregabalin, topical lidocaine patches all to decrease
pain

Pretest
Impaired position sense: diabetes, spondylosis, alcoholism, tabes dorsalis

Increased T2 signal in periventricular distribution is plaques of demyelinating

disease in MS
Increased T1 signal in the wall of the right carotid artery
Enlarged optic nerve is optic neuritis or infiltration of the nerve by a tumor or other
process
ADEM: acute disseminated encephalomyelitis or MS
-young person with acute/subacute onset of progressive weakness; MRI shows spots
of white

Intention tremor: cerebellum

choreiform movements from hepatolenticular degeneration: Wilsons and

Huntingtons disease

pons: characteristic conjugate deviations of the eyes- rhythmic downward

movements ocular bobbing
cervicomedullary junction (meningioma): down-beating nystagmus

increased ICP: blurring of optic disc margins, elevation of the disc because of
swelling
subacute onset of morning headaches with focal neurological findings
pigmentary degeneration of the retina: infections (congenital toxo, CMV, retinitis
pigmentosa)
Hollenhorst plaques: cholesterol and calcific deposits in the retinal arterioles due to
atheroembolism to the eye

Calorics: COWS nystagmus

Sympathetic path: hypothalamus, travels down through lateral aspect of brainstem,

synapses in intermediolateral cell column of spinal cord, exits spinal cord at level of
T1, synapses again in the superior cervical ganglion, travel along the surface of
common caroti and internal carotids until branches leave along the ophthalmic
artery to the eye.
Painful Horner after vigorous activity= carotid artery dissection
Lesion III would be expected to cause ptosis with ipsilateral mydriasis; ptosis
is much more severe in CNIII palsy than in Horner (CNIII- levator palpebrae/main
levator; sympathetics- Muller muscle/accessory role)

CN7 palsy regeneration can cause aberrant regeneration with misconnections-

Habit spasm/idiopathic tic- movement disorder not associated with facial
weakness

Sudden onset of headache- SAH

If negative, then LP (RBC, xanthochromia, increased OP)

Tentorium ceerebelli=fold of meninges, so it is common site for meningiomas

Calcified masses- hyperdense w/o contras

Vascular lesions- dense with contrast

Dermatomyositis: periungal telangiectasias; Dx- CPK, EMG, muscle biopsy

Polymyositis- necrosis of muscle fiber segments with macrophage and
lymphocyte infiltration
Dermatomyositis- perifascicular muscle fiber atrophy, inflmmatroy infiltrate in
the perimysial connective tissue, rather than muscle fibers themselves
 Inclusion body myositis- like polymyositis + rimmed vacuoles

GBS- ascending paralysis with preserved sensation

- High protein, xanthocrhomic 2/2 high protein; extremely high protein
(extremely viscous); glucose normal, WBC normal, ICP normal

SAH- High protein and xanthochromia in CSF suggests blood; WBC elevated because
subarachnoid blood irritates and produces chemical meningitis, normal glucose,
very high RBCs, slight increase in pressure

HSV- high protein, normal glucose, high WBC, slightly high RBCs,

MS- slightly high protein, slightly high glucose, slightly high WBCs, normal pressure,
increased IgG

Psuedoumor cerebri- increased opening pressure; no risk of herniation

CVA
Atherosclerosis is most common cause of MCA infarct (systemic emboli to MCA)
Posteroventral nucleus of lateral thalamus- contralateral numbness and tingling,
with possible eventual paradoxical pain (thalamic pain syndrome)
Dysphagia and dysphonia- CN9,10

Nucleus ambiguous- ventrolateral medulla CN9, CN10innervate striated muscles

of larynx and pharynx, preganglionic parasympathetic supply to thoracic organ
Nucleus solitaries- CN7,9,10 for visceral sensation

Lateral medullary syndrome (Wallenberg)- Vertebral artery mainly, sometimes PICA

Cerebral amyloig angiopathy: MCC of lobar/intracranial hemorrhage in elderly

patiens without HTN; usually >70 yo, multiple cortical hemorrhages

IVDA with CVA: aneurysm rupture from mycotic aneurysm during exertion; old
lesions may be hematomas (especially severe headaches suggest SAH 2/2
aneurysm rupture)
Mycotic here suggests appearance, and is more likely to be from strep or
staph infection associated with endocarditis rather than fungal origin
Aneurysm develops from an infected embolus that came from damaged heart
valves and lodged into the arterial walls of the brain
IVDA->endocarditis->septic emboli->arterial wall damage->aneurysm->SAH

Post-hemorrhagic seizure
Tx- anticonvulsant; Levetiracetam (Keppra)>lamotrigine because faster onset
Post-ictal weakness (Todd paralysis)
-can last days; not suggestive of bleeding or additional cerbrocortical damage, but
CT scan to rule out these possibilities is appropriate
Ex- possibly 2/2 to neuronal exhaustion or depletion of glucose

Sturge-Weber syndrome
-facial angiomas, leptomeningeal angiomas, choroidal angioma of eye
-mental retardation +/- hemiparesis or hemiatrophy contralateral to nevus
- intracranial angioma is unlikely if nevus does not involve upper face

Charcot-bouchard aneurysm: lenticulostriate arteries; caudate nucleus, thalamus,

pons, cerebellum (dentate nucleus)

Amaurosis fugax: recurrent transient blindness, usually 2/2 internal carotid artery
disease
- Symptomatic extracranial carotid artery disease has high likelihood of
developing strokes
Ex- embolism to central RETINAL ARTERY or one of its branches; Hollenhorst
plaques (cholesterol emboli)
Dx- Doppler US
Tx- carotid endarterectomy for symptomatic disease; carotid angioplasty with
stneting is an alternative; aspirin for post-op

Frequent errors in finding words, but follows complex commands somewhat

inconsistently, inability to repeat
Conduction aphasia (impaired repetition)
Brocas affects writing as well

Protracted hypotension: watershed infarct; transcortical mixed aphasia

-poor recovery of language abilities
Impaired object naming and repetition; comprehension preserved (brocas-left
inferior frontal gyrus)
Anomic aphasia can often have word-finding deficit- diffuse brain dysfunction

Primary generalized epilepsy of childhood: atonia

dx- 3Hz spike and wave pattern
1-2 Hz is severe neurological dysfunction and symptomatic generalized epilepsy

Seizure:
Tx- levetiracetam
Status epilepticus
Tx- lorazepam>diazepam (equally fast onset but more fat soluble so stays in
brain longer); phenytoin for ppx
- Phenytoin is used as backup, and can cause cardiac arrthmia if given too
quickly; fosphenytoin has 2 advantages- water-soluble prodrug causes less
infusion site reactions, and it can be administered IM if IV is difficult
- Phenobarbital: SE is hypotension and respiratory arrest
- Diazepam and clonazepam can depress blood pressure and other
autonomic functions

If seizure is already clinically diagnosed by history, then MRI should be done

Alcohol w/d seizure (no prodrome)- within 1 day; DT is 2-4 days

Lennox-Gastaut syndrome- multiple seizure types, 1-2 Hz generalized spikes on

EEGs
 -often with history of infantile spasms (West syndrome)- paroxysm flexions of
the body, waist, or neck with wildly crazy EEG (hypsarrhythmia)
Landau-Kleffner syndrome- loss of language function and abnormal EEG during
sleep
Juvenile myoclonic epilepsy
Febrile seizure- tonic clonic

Post-traumatic seizures- anticonvulsants can reduce incidence of early (but not late)
posttraumatic seizures

Sclerotic right hippocampus- MCC of intractable complex partial seizures

Tx- neurosurgery; poor response to medications

West syndrome 2/2 tuberous sclerosis: generalized seizure, recurrent spasms,

hypsarrhthmia, retardation
Tx- ACTH

Jacksonian march (sequential seizure)- focal seizure that often secondarily

generalized

Myoclonic seizures (often apart of benign juvenile myoclonic epilepsy): similar to

sleep myoclonus, but happens when person wakes up rather than falling asleep.
Jerks can be triggered by light flashes or loud sounds. More than half of BJME have
generalized tonic-clonic seizures as well as myoclonic seizures

Epliepsia partialis continua: persistent focal motor seizure activity (focal motor
status epilepticus)- distal hand and feet most frequently affected; seizure can
persist for months; poor response to therapy

Peripheral neuropathy
Isoniazid- pyridoxine sequestration
Pyridoxine overdose- peripheral neuropathy, hypochondriasis or OCD

Lyme neuropathy (noncompressive sciatica): acute onset painful radiculopathies

and peripheral neuropathies

Lead poisoning- painless neuropathy, esp. radial nerve (wrist drop)

Thalium- hair loss, stupor, GI, seizures, headaches, painful symmetric sensory
neuropathy
Manganese- parkinsonism
Mercury- painful neuropathy

Mononeuropathy multiplex- weakness in multiple muscles and limbs without pattern

Ex- MCC is diabetes

Freidreich disease:
-degeneration in the spinal cord (spinocerebellar tracts)
GBS emergent treatment is plasma exchange
LE treatment is 3,4-Diamnopyridine

Parsonage-Turner syndrome
Ex- acute brachial plexopathy; neuralgic amyotrophy
Sx- acute onset of pain in neck, shoulder, or upper arm; then 3-10 days later,
there is rapid evolution of weakness affecting the proximal upper extremity, with
loss of reflexes; rarely, respiratory muscles are affected
Sensory loss also occurs

Riley-Day disease- familial dysautonomia

- affects mainly Jewish children; loss of tears on crying, corneal ulceration, and
absent pupillary reactivity; poor temperature regulation, excessive
perspiration, abnormal blood pressure control, dysphagia, recurrent vomiting,
and gastric and intestinal dilation; absence of papillae on tongue
CIPD~ GBS but slowly progressive and remitting rather than acute onset and rapid
resolution
-polyradiculoneuropathy (affects proximal portions of the nerves where they exit the
spinal cord at the root level)
-affects nerve roots, so affects hands and feet and proximal and distal
- Dx- EMG- multifocal conduction blocl, slowing of nerve conduction, loss of
amplitude of muscle action potentials; CSF- elevated protein to 260 (suggestive of
inflammatory process), but no cells

Porphyric polyneuropathy: acute intermittent porphyria

- recurrent attacks of abdominal pain, gastroparesis and constipation (2/2
autonomic neuropathy), psychosis/confusion, axonal motor neuropathy (weak and
areflexic, with intact sensory), and autonomic instability
- seizures and SIADH may also occur
- Ex- attacks can be due to porphyrinogenic drugs like baribiturates, phenytoin
sulfas, and estrogens
Tx- IV hematin if supportive measures are not enough

Charcot Marrie Tooth- Hereditary motor and sensory neuropathy (HMSN) and
peroneal muscular atrophy (PMA)/stork leg/inverted champagne bottle
- Progressive loss of muscle tissue and touch sensation
- Foot drop, hammer toe, high arched feet/flat arched feet, painful on/off
spasmodic muscular contractions
- Involuntary teeth grinding and squinting

Meralgia paresthetica
- pain or irritating sensation in anterolateral aspect of thigh due to irritation to
femoral cutaneous nerve

Critical illness neuropathy

- flaccid muscle weakness diffusely including diaphragm in critically ill patients

Sarcoid: optic atrophy and facial nerve palsies; may be confused with MS early on
Thiamine deficiency- painful neurpathy in patients with alcoholism,
Spinal shock: transient phenomenon that results from damaged fibers of upper
motor neurons, resulting in spasticity (from overexageration of normal stretch
reflexes), may also occur with cerebrocortical injuries

Fractures of vertebral body without trauma must be evaluated for malignant

processes (metastatic carcinoma, multiple myeloma, and unsuspected
osteomyelitis)

Disrupted CSF flow (ex. 2/2 thoracic disk herniation) can lead to cyst lesion (syrinx),
which may expand and compress anterior horn of spinal cord leading to lower
motor neuron
Contusions are common causes of syrinx formation, as damaged tissue is
removed; ischemia must be substantial and persistent enough; demyelination does
not

Both dorsal and ventral interosseous muscles are innervated by ulnar nerve
PAD-DAB: 4 dorsal and are bipennate; 3 ventral and are unipennate
Transverse myelitis 2/2 schistosomiasis: ovum in granuloma

S. mansoni endemic in Puerto rico and south America; tx: praziquantel

S. japonicum no spine

Ischemic spinal cord: bruning and occasionally shooting pain; does not extend into
his legs, but sometimes has associated weakness; exertion like walking will result in
pain in legs and tingling sensation in feet
Spinal cord infarction: CSF normal except elevated CSF protein
Main source of blood to spinal cord is aorta (vertebral and hypogastric
arteries)great anterior medullary artery (of Adamkiewicz) that supplies the
anterior median spinal artery
Paraplegia with loss of sensation and temperature below a level: anterior spinal
artery syndrome
-
Chorea gravidarum: Ex- dramatic change in hormonal environment of brain during
pregnancy; sx- crapid and fluid, but not rhythmic, limb and trunk movements

Post-encephalitis parkinsonism: chorea then parkinsonism

Huntingtons disease: widening of lateral ventricles; dopaminergic drugs can

unmask chorea because theres neuronal death and the remaining dopaminergic
receptors become more sensitive to dopamine

Tremor goes away with concentration- Parkinsonian tremor

Stimulate globus pallidus, STN, and thalamus

Meige Syndome: focal dystonia of the face and neck; Tx- botox

Spasmodic torticollis: focal dystonia of the neck that may be painful and produce
hypertrophy standing and walking worsens the contractions, trick/geste/touching
the chin or resting the head against a pillow can reduce the spasms; tx-
trihexyphenidyl, unresponsive to botox
Essential tremor: tx- alcohol, beta blokers, primidone

Hepatolenticular degeneration- (Wilsons disease) hard liver, enlarged spleen, renal

tubular acidosis/metabolic acidosis, heart and lung damage; tremor and rigidity,
reduced blink, atrophy of putamen and globus pallidus

Primary hyperparathyroidism: esp if mentioning of pseduogout; excessive

sleepiness, slowing of movements, mild depression, and proximal muscle weakness

Oligoclonal bands in csf: MS, syhphilis, lyme, SSPE

MS spams: Tx- baclofen for urgency/cyclophosphagmide, thizanidine, benzos

Sx- worse with warmth (hot shower, exercise)

Neuromyelitis optica: bilateral optic neuritis associated with transverse myelitis

Acute disseminated encephalomyelitis: after infectious disease, headache and neck

stiffness; fever followed by deteriorating cognitive function, disorientation, lethargy,
and increasing unresponsiveness
Dx- damage to small blood vessels and perivascular tissues in the white
matter of the cerebrum; MRI or CT shows rapidly evolving white matter damage
associated with high ESR and increased CSF pressure with RBCs and WBCs and
protein. Normal CSF glucose
Optic neuritis: expansion of blind spot
Centrocecal scotoma- impingement of blind spot on central vision
Leber optic atrophy: progressive loss of vision over years, often involves centrocecal
scotoma; mitochondrial DNA mutation

Adrenoleukodystrophy- adrenal dysfunction in association with progressive

degenerative disease of the white matter; X-linked disorder of ATP-binding
transporter in the peroxisomal system for long-chain fatty acid metabolism leading
to accumulation of fatty acids in adrenal cortex
Sx- spastic paraparesis, problems with bladder and bower control, and
sensory disturbances in the legs

Central pontine myelinolysis: rapid correction of hyponatremia

Similar symptoms as Wernicke encephalopathy, but if you give thiamine
before glucose it is enough to prevent Wernickes
-
Eye
Retinitis pigmentosa- MC retinal degeneration
- deisease of retinal receptors and pigment cells; pallor of optic disc is a late
finding
congenital cataracts- common cause of opacification of light/absence of red reflex
(2/2 scar from retinopathy of prematurity, retinoblastoma)

central scotoma
- acute: methyl alcohol
- subacute/chronic: isoniazid, ethambutol, streptomycin
papillitis vs. papilledema
papillitis: substantial visual loss, pain, sensitive to light pressure; often an
early sign of MS

tunnel vision: 2/2 conversion disorder or malingering; sx- size field remains the
same even as test screen is removed farther away
concentric constriction: associated with optic atrophy (from things like
neurosyphilis) will result in enlarged perceived area as test screen is moved farther
away

MS: bladder incontinence (corticospinal tract),

Dx- MRI

HTN retinopathy
Dx- fundoscopy showing segmental narrowing of arterioles, arteriolar
straightening, and AV-compression

Diabetic retinopathy
Dx- fundoscopy showing retinal microaneurysms (small red dots)
CN6>CN3>CN4 palsy

Abducens dysfunction is likely to due to increased ICP like neoplasm or direct

damage to the brainstem;

Gadenigo syndrome: osteopyelitis of the petrous pyramid, affecting abducens and

trigeminal nerves (they pass close to the petrous bone); chronic ear infections may
extend to the petrous pyramid
Sx- kid with facial pain and blurry vision
Fourth cranial nerve is most likely to be damaged from direct hit to front of face,
and will induce a slight head tilt to compensate for impaired intorsion of the eye
Fourth cranial nerve is most likely to be involved in VZV because it shares a cranial
nerve sheath with the V1 of trigeminal nerve. CN3 and CN6 are less commonly
ivoled

Nystagmus-inducing drugs: alcohol and barbiturates

Ocular bobbing: pontine or cerebellar damage; rapid downward deviation of both

eyes followed by slow upward conjugate eye movements

Cavernous sinus thrombosis: proptosis and pain, with impaired acuity and
profoundly disturbed retina

Diphtheritic polyneuropathy: opthalmoplegia, unilateral

Adie tonic pupil: benign tonic pupillary dilation in otherwise healthy young women,
may occur in association with absent tendon reflexes; if one eye then consider local
trauma, if both pupils then consider drug use

Acute monocular blindness: MCC is ischemic optic neuropathy (anterior ischemic

optic neuropathy(+);
 Symptoms may progress over several days,; field is usually inferior, with
involvement of central vision, leading to impairment of vision and acuity. Up to 1/3
of people, the opposite eye may become involved soon after
PF is HTN and DM; arterial occlusion is posterior ciliary artery, which supplies
the optic nerve
Exclude giant cell arteritis

MS would cause loss of vision for entire eye; other symptoms can be ataxia,
paraparesis, and dysarthria

L Meningioma: L central scotoma; R upper R vision loss (upper quadrant

preferentially affected)