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400 Cards in this Set

  • Front
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Strange smell followed by LOC, jerking movements, and loss of bowel/bladder control
Simple partial seizure w/ secondary generalization
Strange smell, automatisms, LOC
Complex partial seizure
Rx for cluster headache (2)
100% O2
SubQ triptans
Prophy for cluster headaches (3)
Verapamil
Lithium
Ergotamine
Headache that awakens pt from sleeping and whoosing in ears
Pseudotumor
Rx for pseudotumor
Acetazolamide
Possible MRI findings in pseudotumor
Empty sella
Slit-like ventricles
Why is an MR venography obtained in pts with potential pseudotumor?
Cerebral venous sinus thrombosis can present similarly (and also has elevated opening pressure), so must be excluded
Pathophys of fever
Cytokine activation
Athlete in heat w/ temp >105, AMS, dehydration, hypotension, tachy
Heat stroke
Pathophys of heat stroke
Failure of thermoregulatory center
Large blood with no RBCs on UA
Rhabdomyolysis (due to muscle breakdown)
Pathophys of malignant hyperthermia
Uncontrolled efflux of calcium from sarcoplasmic reticulum
Inadequate fluid and water replacement during physical activity
Heat exhaustion
Cool skin, low BP, confusion, and muscle spasms after intense physical activity
Heat exhaustion (thermoregulatory apparatus is intact)
Rx for heat exhaustion
Remove from heat to prevent hyperthermia
Most common presenting sign of craniopharyngioma (other than bitemporal hemianopsia) in children vs. adults
Children: growth retardation
Adults: sexual dysfunciton
Rx for subdural hematoma w/ midline shift
Emergent neurosurg consult for hematoma evacuation
Cause of foot drop
Inability to dorsiflex the foot
What is festinating gait and what disease can it indicate?
Slow onset of movement followed by short shuffling steps

Parkinson's
3 causes of foot drop
Neuropathy
L5 radiculopathy
Trauma to common peroneal nerve
Rx for Lambert Eaton syndrome
Plasmapheresis and immunosuppresants
Pathologic findings in MS
Perivascular inflammation and destruction of myelin in white matter
Two CSF findings of inflammation
Oligoclonal bands and elevated IgG index (ratio of IgG to albumin in CSF/ ratio of IgG to albumin in serum)
Differentiate btwn primary and secondary progressive MS
Latter was relapsing-remitting at diagnosis
4th MS subtype
Progressive relapsing
6 most common initial MS Sx
Weakness in 1+ limbs, optic neuritis, sensory Sx, diplopia, vertigo, disturbance of micturition
What is Lhermitte's sign
Electric sensation passing down back and into limbs provoked by neck flexion
Most common disease modifying agent used in MS and three brands
Interferon beta
Avonex and Rebic (1a) and Betaseron (1b)
3 other disease modifying agents
Glatiramer acetate (Copaxone): synthetic myelin analogue

Natalizumab (Tysabri): Ab against T-cell adhesion molecule alpha-4 integrin

Mitoxanitrone (Novanrone): chemo agent
Main toxicity of mitoxantrone and consequent dose limit
Cardiotoxicity, 3 yrs
Acute MS relapse Rx
Glucocorticoids
No meds currently approved for which MS subtype?
Primary progressive
Common drugs for MS side effects
Anti-spastics
Migraine pathophys
Trigeminovascular activation, inflammation, and cortical spreading depression
When is the "worst headache of my life" not an emergency?
When only quantitatively, not qualitatively
Empiric Abx for meningitis and what to add if suspicion v. high
2g ceftriaxone, 1gm vanc +/- amp in immunocompromised pts (for Listeria)

If suspicion high, add glucocorticoids
When should an LP be done before CT?
If CT will take >1hr
When can Abx be d/c
No WBCs in CSF or negative cultures after 48hrs
CSF in viral meningitis
WBC <100, lymphocytic, nml glucose
Suspected SAH
Spin CSF! Traumatic blood will aggregate at bottom so appears clear; SAH: xanthrochromia
Rx for SAH
Neurosurg consult to clip or coil aneurysm w/in 2 days

Nimodipine
Rx for temporal arteritis
60-80mg prednisone daily; if biopsy positive, continue for 6-8 wks then taper
Intention tremors are due to damage where?
Cerebellar hemisspheres
Sleep has what effect on Parkinsonian tremor during sleep
Causes it to cease
Possible eye side effect of phenytoin
Nystagmus
Other toxicites of phenytoin
Ataxia, dysarthria, lethargy, impaired judgment
CN cause of hyperacusis
Facial nerve damage --> paralysis of stapedius muscle (middle ear)
Abnormality in tandem gait indicates problem is
Cerebellar
Atrophy and fasciculations of tongue are from
CN XI problems (often brainstem disease, e.g. stroke or ALS)
Loss of ankle jerk reflex
S1 radiculopathy
Calorics: warm water in ear will cause
Nystagmus in both eyes away from stimulated ear
Horner syndrome acutely after vigorous activity
Carotid artery dissection
Most common site of a noncommunicating hydrocephalus
Aqueduct of Sylvius (connection btwn third and fourth ventricle)
Tentorium cerebelli is a common site of origin for what kind of tumors?
Meningiomas
Rx for meningiomas
Surgery + radiation
What is dysdiadochokinesia and what causes it?
Impairment of rapid alternating movements

Cerebellar damage, e.g. MS/cerebellar tumors
Frequency of alpha waves and when they are emitted
8-13Hz
Relaxed adult with eyes closed
CSF in bacterial meningitis
Elevated protein, few RBCs, elevated opening pressure, milky fluid, nml gamma globulin, high WBCs, very low glucose
CSF in Guillain Barre
Normal except v. high protein and yellow (from high protein)
3 CSF findings in SAH
Xanthochromia/ elevated RBCs (old), high protein (from broken down blood), elevated WBCs (chemical meningitis from irritation from blood)
Elevated gamma globulin (e.g. elevated %protein without elevation in protein content) is CSF finding typical of
MS
CSF in viral meningitis
Some RBCs/WBCs, elevated protein, nml glucose
Class of drugs used in secondary progressive MS
Immunosuppressants, e.g. cyclophosphamide (as well as typical MS drugs)
Risk of carbamazepine and monitoring required
Aplastic anemia
CBC
Surgical options for refractory trigeminal neuralgia
Surgical gangliolysis
Suboccipital craniectomy for decompression of trigeminal nerve
Rx for pseudotumor with progressive visual field deficits
Shunting or optic nerve sheath fenestration
Drugs used to control severe HTN in ischemic stroke
CCBs (nicardipine)
ACEIs
(not aiming for tight control)
What is torticollis?
Focal dystonia of SCM
What is athetosis and what can it indicate?
Slow, writhing movements of hands and feet

Huntington's
HTN with focal neurologic deficits
Spontaneous intraparenchymal hematoma (most commonly in the basal ganglia)

SAH would have headache, usually no focal findings
4 common sites of spontaneous intracranial hemorrhage
Basal ganglia, cerebellum, thalamus, pons
Required for diagnosis of dementia
Functional impairments
2 first line drugs for absence seizures
Ethosuximide
Valproic acid
Huntington's that develops in childhood w/ more bradykinesia and rigidity + seizures, myoclonus, dystonia
Westphal variant
Anatomy of Huntington's disease
Neostriatum (atrophy of caudate and putamen)
2 causes of Sydenham chorea
Pregnancy, rheumatic fever
Rx for chorea
Haloperidol
Sustained muscle contractions, twisting, and repetitive movements since childhood
Primary generalized dystonia
What is opisthotonos
Rigid spasm of the body w/ back fully arched and heels/head bent back
Hints that dystonia may be secondary
Other neuro problems, hemidystonia, dystonia at rest or incl. speech at onset
3 Rx options for dystonia
Parkinson's drugs
Targeted Botox/ baclofen
DBS of GPi
Inheritance of primary dystonia
Most common type (DYT-1) is AD
Sensory problems in Parkinson's
Pain, loss of smell
Other non-motor problems in Parkinson's
Dysarthria, stuttering, dementia
Pathologic finding in PD
Loss of pigment in substantia nigra, striatal dopamine deficiency (>70% when Sx emerge, >90% at death)
Worry about other diseases if Parkinson's symptoms +
Rapid progression
UMN signs
Incontinence
Cerebellar signs
Unilateral PD
Corciobasal ganglionic degeneration
PD Sx w/ autonomic instability
Multiple system atrophy
PD Sx w/ inability to look down and frequent early falls
Progressive supranuclear palsy
Rx options for Parkinson's (5)
1) Levodopa + carbidopa (Sinemet) +/- COMT inhibitors
2) Dopamine agonists (pramipexole, ropinirole, bromocriptine)
3) MAO-B inhibitors (selegiline, rasagiline)
4) Anticholinergics for tremor
5) Amantadine for tremor and levodopa dyskinesia
Progressive ataxia + dysarthria and LMN/UMN signs
Spinocerebellar ataxia (AD)
MRI findings of SCA
Cerebellar atrophy
Rx for tardive dyskinesia
Benzos, baclofen, Vita E
Localization of flaccid quadriparesis
CST in upper cervical region
Lack of DTRs or rectal tone
Spinal shock
When are steroids indicated for SCIs?
Adults w/ incomplete SCI if w/in 8 hrs (IV methylprednisone)
Kids <9 w/ traumatic injury always need
Cord imaging
Transient LOC after a head injury is often due to damage where?
Ascending reticular activating system
3 grades of concussions
Grade 1: Sx < 15 min
Grade 2: Sx >15 min
Grade 3: LOC
Sx of postconcussive syndrome
Headaches and dizziness
Also: irritable, depression, insomnia, cognitive
Return to play guidelines for each of the concussion grades
Grade 1: 15 min after return to baseline
Grade 2: after 1 wk
Grade 3: after 1-2 wks depending on length of LOC
(all are longer if 2nd concussion)
DDx for stroke-like weakness
Seizure w/ Todd's paralysis
Complicated migraine
Pure motor or sensory strokes localize where?
Lacunar strokes
Localization of stroke w/ aphasia, neglect, agonisa, apraxia
Anterior (carotid) circulation
Localization of stroke w/ diploplia, vertigo, homonymous hemianopia, crossed findings
Posterior (vertebrobasilar) circulation
For non-tPA candidates w/ stroke, give
ASA
SAH often has what recent Hx
Moderate headache (sentinel bleed)
60% of SAHs occur during
Sex, trauma, emotional/physical strain, defecation
2 common systemic complications of SAH
Hyponatremia and ECG changes (QT prolongation, T wave inversion)
What is acute communicating hydrocephalus?
Obstruction of subarachnoid granulations due to blood --> enlarged ventricles --> headache, vomiting, blurry vision, somnolence, syncope
2 diseases related to aneurysms
Fibromuscular dysplasia
Polycystic kidney disease
2 drugs that cause SAH
Cocaine
Amphetamines
# of grades of SAH, which is best
5 (based on clinical Sx)
1 is best, 5 is worst
Rx for SAH
Angiography w/ coiling > clipping
Triple H (HTN, hypervolemia, hemodilution)
Nimodipine (CCB)
Acute change in LOC 1 wk after SAH: imaging algorithm?
1st: CT for hydrocephalus (to see if need ventriculostomy)
Then, diffusion-weighted MRI for ischemia 2/2 vasospasm; confirm with angiography
Risk with a choke hold
Carotid artery occlusion --> ischemic stroke
3 causes of carotid artery dissection (tear in wall) (general/pathologically)
Luminal obstruction
Thromboembolic complications
Pseudoaneurysm formation
Stroke symptoms + Horner's may indicate what etiology of the stroke?
Carotid dissection
AV malformations are associated w/ what type of stroke?
Intracerebral hemorrhage
When are carotid dissections stented?
Only in pts who show no vessel recanalization after 3-6mo
What is moyamoya disease?
Idiopathic noninflammatory cerebral vasculopathy; causes progressive occlusion of large arteries at circle of willis (arteries hypertrophy in response to chronic ischemia)
Difference btwn a partial and generalized seizure
Partial only relates to one of the cerebral hemispheres, whereas generalized involves both
Should you treat a first-onset adult seizure?
Controversial, but usually not; 75% won't recur (only 10-25% go on to epilepsy), and while treatment decreases relapse rate, no evidence that it alters prognosis of epilepsy
First line for tonic-clonic seizures
Valproate
Most common seizure aura
Sensation of abdominal discomfort
2 brain areas that must be involved in complex partial seizures
Basal forebrain and limbic areas
3 ways to distinguish absence seizures from complex partial seizures
Absence have no aura, no automatisms (usually), and no postictal confusion
Bizarre automatisms often indicate seizures where?
Frontal lobe
3 classes of auras, based on seizure location
Abdominal sensation, fear, deja vu: temporal lobe

Electrical sensation, tingling, numbness: parietal lobe

Visual changes: occipital lobe
How much folic acid should women of childbearing age on AEDs be prescribed?
4mg daily (to prevent NTDs)
What is catemenial epilepsy?
Seizure frequeny can double around menstrual cycle (in 1/3 of women with seizures)
Unilateral miosis and ptosis in a pt with a headache and recent neck trauma
Carotid dissection --> Horner syndrome
What is anisocoria?
Unequal sizes of the pupils
What is sick sinus syndrome?
Bradycardia in which the sinoatrial (SA) node isn't working properly
Cough or micturition syncope imply what problem?
Decrease in venous return
Rx for sick sinus syndrome
Implantable pacemaker
Rx for orthostatic hypotension (3 first line and 2 second line options)
Avoid hypovolemia, electrolyte imbalance, and excess alcohol

Increase salt intake, start fludrocortisone
Dysautonomic causes of orthostatic hypotension (7)
Diabetic neuropathy
Amyloidosis
Syphilis
SCI
Syringomyelia
Alcoholic neuropathy
AIDP
Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) is also known as
Guillain Barre syndrome
Why is video EEG monitoring needed for pseudoseizures?
1-3% have organic seizures as well, need to r/o
Pt treated for temporal arteritis who develops proximal muscle weakness afterwards likely has
Steroid-induced myopathy! Especially if ESR normal (and already received steroids, the treatment), unlikely to be PMR
Contralateral hemianesthesia/ dysesthesia can be caused by a stroke where
VPL nucleus of thalamus (thalamic pain phenomenon)
HIV pt with multiple demyelinating, non-enhancing lesions w/ no mass effect + hemiparesis and disturbance of speech, vision, and gait
PML
Toxo lesions are usually where?
Basal ganglia
CSF finding specific for primary CNS lymphoma
EBV DNA
Common early side effects of sinemet
Somnolence, confusion, hallucinations (same w/ dopamine agonists)

Dyskinesia/ motor fluctuations later
Common side effect of amantadine
Ankle edema
Livedo reticularis
Cape-like pain and temp loss
Syringomyelia
Pathophys of syringomyelia
Cavitary expansion of spinal cord
How can anticholinergic meds cause headache/ retro-orbital pain?
May precipitate acute glaucoma
Rapidly progressive ascending paralysis with loss of DTRs but no fever or sensory abnormalities
Tick-borne paralysis
CSF in tick-borne paralysis
Normal
Rx for tick-borne paralysis
Removal of tick
New headache (around eye) and halos around lights + non-reactive, dilated pupil and erythematous eye
Acute angle closure glaucoma
AMS, tachycardia, coagulopathic bleeding, temp >40.5C (105F)
Heat stroke (often exertional)
Neurogenic fever is often caused by abnormalities where?
Hypothalamus (thermoregulatory mechanism)
Down and out gaze
CN III neuropathy
Best test for acute glacuoma
Tonometry
Pronator drift indicates
UMN disease (exaggerates the strength of the pronators over the supinators)
Cortical location of motor vs. sensory findings
Motor: frontal lobe
Sensory: parietal lobe
Dysarthria + clumsy hand syndrome
Lacunar stroke
Biggest risk factor for lacunar strokes
HTN
Pure motor hemiparesis or ataxia-hemiparesis
Lacunar stroke (posterior internal capsule)
Pure sensory stroke
VPL nucleus of thalamus
Drug approved for ALS and mechanism
Riluzole

Glutamate inhibitor
Findings in Wernicke-Korsakoff syndrome (3 + 2)
Ataxia, ophthalmoplegia, confusion (Wernicke's)

Plus confabulation and amnesia (Korsakoff's)
Most prominent finding in NPH
Broad-based gait and shuffling, some bradykinesia
Most common cause of myesthenic crisis
Infection
Rx for myasthenic crisis
Intubation and withdrawal of anticholinesterases for several days (may have induced excess cholinergic Sx)
Rx for normal pressure hydrocephalus
Repeated LPs; if improve, VP shunt
Ipsilateral ataxia (pt falls toward side of lesion) and weakness
Cerebellar tumor
Hemiparesis, hemi-sensory loss, homonymous hemianopsia, eyes deviated away from paralytic side
Putamen hemorrhage
Most common site of hypertensive hemorrhage
Putamen (next to internal capsule, so hemiparesis is common)
Presentation of subarachnoid hemorrhage
Severe headache WITHOUT focal neuro signs
Rx for exertional heat stroke
Evaporation cooling (spray naked pt with lukewarm water and running fans to circulate air)
Common electrolyte abnormality in subarachnoid hemorrhage
Hyponatremia (from SIADH and excess ANP --> cerebral salt wasting)
Pick's disease
Frontotemporal dementia (may have FH)
Where is the blood on CT in a pt with subarachnoid hemorrhage
Sulcai and cisternae
Child with staring spells, automatism, and post-ictal confusion
Complex partial seizure (NOT absence if post-ictal)
Difference btwn typical and atypical absence seizures
Atypical last longer
What is Lennox-Gastaut syndrome?
Childhood seizures of multiple types, impaired cognitive fxn, and slow spike-and-wave activity on EEG
Decreased strength and sensation only in the LEs and + Babinski
Spinal cord compression! (UMN signs as well as LMN; no UMN signs in GBS)
Erythema migrans, headache, arthralgias, myalgias
Lyme disease
Work-up of amaurosis fugax
Carotid duplex (most common site of emboli)
2 things that can predispose to pseudotumor
Isotretinoin or Vitamin A toxicity
When can't you give beta blcokers for essential tremor?
Pt with bradycardia or severe COPD
What is primidone
Anticonvulsant (converts ito phenylethylmalonamide and phenobarb)
Risk of primidone
Acute intermittent porphyria (abd pain, neuro and psych abnormalities)
Test to diagnose acute intermittent porphyria
Urine porphobilinogen
Low back pain, bowel/bladder dysfunction, saddle anesthesia, sciatica, LE sensory/motor loss
Cauda equina syndrome
How to distinguish cauda equina syndrome from disease in lumbar spine?
Latter has UMN signs; cauda equina only has lower motor neurons
Cauda equina syndrome indicates disease where?
Spinal nerve roots
Ring-enhancing lesion in brain + fluid collection in maxillary sinus
Brain abscess (usually strep or anaerobes) 2/2 maxillary sinusitis
Acute occipital headache, repeated vomiting, gait ataxia, HTN
Cerebellar hemorrhage
Pt is very concerned about their impaired memory
More likely to be pseudodementia than dementia
What normal neuro finding can brain dead patients still have?
DTRs (spinal cord finding)
Parkinsonism + autonomic dysfunction
Multiple system atrophy
What is Shy Drager syndrome?
Multiple system atrophy
Rx for multiple system atrophy
Volume expansion (salt, fludrocortisone, alpha agonists)
Most common site of ulnar nerve entrapment
Elbow
Systemic condition that predisposes to carpal tunnel syndrome
Hypothyroidism
Ventricles in NPH
Enlarged
Side effects of pyridostigmine or neostigmine for myasthenia
Abdominal cramps, fasciculations, muscular weakness
Why is neglect usually on the left?
Involves the right parietal lobe (non-dominant)
Bilateral trigeminal neuralgia may indicate
MS
2 common types of migrainous aura
Visual and sensory
Rx for persistent postspinal headache
Epidural blood patch (pts blood injected into epidural space at site of initial tap)
Mechanism of triptans
5HT-1D serotonin receptor agonists
Contraindication for triptans
Hx of CAD or HTN
Hemiplegia or blindness as aura
All pts with chronic daily headache need
MRI
What to avoid to Alzheimer's drugs
Don't start 2 at once
Sense impaired in early AD?
Olfaction
Risk factors for AD
Apolipoprotein E type E4
Degeneration of AD commonly is where?
Nucleus basalis of Meynert (cholinergic cells project there)
Motor parkinsonism w/ executive dementia and visual hallucinations
Lew body dementia
Occipital lobe hypometabolism
Lew body dementia
Atrophy of midbrain
Progressive supranuclear palsy
Temporal lobe atrophy and parietal temporal hypometabolism
Alzheimer's
Paresthesias in hands and feet, vibratory loss, ataxia, and mental changes
B12 deficiency
Pathology of subacute combined degeneration of spinal cord
Edema and destruction of myelin
Sensory loss + spastic paresis
Subacute combined degeneration (lesions in dorsal column and lateral CST)
2 other lab values in B12 deficiency
Increased homocysteine, increased methylmalonic acid
Slowed velocity on NCS means diagnosis is not
Multiple sclerosis (b/c is a CNS, not PNS problem)
3 other diseases associated w/ optic neuritis
Sjogrens, GBS, HIV
Visual test findings in MS
Prolongation of visual evoked responses
ADEM often follows (2 things)
Infection
Vaccination
ADEM can progress to?
MS (25%)
Hyperacute ADEM
Acute necrotizing hemorrhagic encephalomyelitis
Finding in ADEM
Perivenular demyelination (inflammatory myelinopathy)
Rx for ADEM
Usually nothing: steroids, PLEX, or IVIg if needed
Don't confuse ADEM with this disease, and how to differentiate
GBS (prior infection, weakness, urinary incontinence in both, but HEMIparesis and HEMIsensory is ADEM)
If suspicion for meningitis is v. high, when must a CT still be performed first?
Focal neuro findings or papilledema
Most common cause of viral meningitis
Enteroviruses
Reduced CSF glucose is what value?
<40% serum
MRI in HSV meningitis
Temporal lobe hemorrhage
Suspicion of meningitis w/ skin rash
Neisseria
Rx for neisseria
Penicillin or ampicillin + dexamethasone
Bacterial vs. viral CSF
Bacterial has higher protein and low glucose, PMN predominance
Rx for infantile botulism
ICU (high risk of respiratory depression) and antitoxin human botulism Ig (IV)
Botulism presentation
Descending weakness
Pathophys of botulism
Inhibition of ACh release by toxin --> presynaptic problem
Constipation, hypotonia, respiratory difficulties, CN abnormalities and hyporeflexia
Infantile botulism
Best Dx test for infantile botulism
Stool sample
Foodborne botulism in adults has what Hx?
Antecedent GI disease (N/V/D)
Botulism presentation in a baby with reactive pupils and normal DTRs
Neonatal myasthenia gravis
HIV pt w/ behavioral changes and lack of coordination
HIV-associated dementia
Path findings in HIV-associated dementia
Cerebral atrophy (esp in frontotemporal region) w/ diffuse myelin pallor
Testing for CJD
Periodic short wave complexes on EEG and nml CT
Description of sensory pain in neurosyphilis
Lancinating
4 forms of neurosyphilis
Asymptomatic
Meningovascular
Tabes dorsalis
General paresis
Rx for toxo
Pyrimethamine, sulfadiazine, folinic acid
Prophy for toxo
Bactrim
Unilateral unresponsive pupil, impaired corneal sensation, decreased DTRs in legs
Holmes-Adie syndrome (benign/ normla)
How to differentiate tonic pupil from CN III palsy?
Check accommodation (intact in tonic pupil)
Imaging study for pt w/ papilledema
CT w/ contrast
Increased ICP definition
Pressure >200 (or >250 in obese)
Rx for refractory pseudotumor
LP or VP shunt
Rx for progressive vision loss in pseudotumor
Optic nerve sheath fenestrations
Double vision only on horizontal gaze in a pt with CAD
CN VI palsy (affecting lateral rectus) due to ischemic mononeuropathy)
Covering one eye resolves double vision indicates
Binocular double vision (intracranial problem, vs. monocular which is usually an ophtho issue)
Worsening diploplia on near vision
Medial rectus problem
Rx for ischemic CN VI palsy
Eye patch, prism, botulinum toxin, if no better in 3-6mo: surgery
Unilateral facial paralysis, hearing loss, and blisters on ear
Ramsay-Hunt Syndrome
Ramsay Hunt Syndrome affects which two CNs?
VII and VIII
Facial paralysis caused by a stroke usually spares
Forehead
Epithelial debris from TM that becomes trapped in middle ear
Cholesteatoma
Sensory ganglion of facial nerve
Geniculate ganglion
Ramsay Hunt Syndrome is also known as
Herpes zoster oticus
Inability to raise upper lip on one side
Parotid gland tumor (Isolated facial nerve paralysis in one branch is caused by malignancy until proven otherwise)
Ophtho risk w/ CN VII paralysis
Exposure keratitis (b/c can't blink/tear, need hourly artificial tears)
Test for mitochondrial cytopathies
Serum lactate
Ptosis + CN III palsy
Concern for posterior communicating artery aneurysm
What is MuSK?
Muscle-specific tyrosine kinase (enzyme critical for aggregating ACh receptors)
Test in all myasthenia pts (2)
FVC (to check for respiratory muscle weakness) and TSH
Pathophys of myasthenia gravis
ACh receptor Abs bind ACh receptor, prevent ACh from binding, so post-synaptic potentials aren't enough for an AP
Rx for myasthenia
Immunosuppressants + anti-cholinesterase inhibitors (latter for symptoms only)
Type of herniation that causes ptosis?
Uncal (CN III palsy)
Type of hearing loss w/ cholesteatoma
Conductive
Rx for CIDP
Steroids, IVIg (1st line if is pure motor CIDP), PLEX
When to intubate with GBS
Prophylactic if FVC <15-20mL/kg
GBS findings
Areflexia, proximal LE weakness w/ distal sensory changes and ascending progression
ABs involved in GBS
Anti-ganglioside
Most common GBS subtype
AIDP
Miller-Fisher GBS subtype
Areflexia
Ataxia (out of proportion to sensory deficits)
Ophthomoplegia
CN wekaness > extremities
+ anti-GQ1b (ganglioside) Abs
Acute motor axonal neuropathy (AMAN)
GBS common in kids
Acute motor and sensory axonal neuropathy (AMSAN)
GBS with significant muscle atrophy
Acute pain autonomic neuropathy
GBS in which dysrythmias often contribute to mortality
DDx for acute flaccid paralysis + GI Sx (3)
GBS
Tick paralysis
Botulism (descending)
Timing of GBS
Most severe at 12 days, with improvement from 28-200 days
LP in GBS
Albuminiocytologic dissociation (protein up to 400 but no increase in cell count)
Rx for GBS
5 days IVIg, 10 days PLEX
What are Gottron papules?
Flat-topped raised nonpruritic lesions on MCP, PIP, and DIP joints
What is a mixed headache?
Chronic daily headache with episodic exacerbations
DDx for chronic daily headache (3)
Primary headache disorder
Cerebral venous sinus thrombosis
Increased ICP
Best migraine abortive agents (2) in children
Ibuprofen and acetaminophen are first line
Then sumatriptan nasal spray
Migraine is associated with what other disorder?
Epilepsy (each increases the risk of the other by 2)
Vertigo w/o headache
Migraine equivalent
Diagnostic test for muscular dystrophy
Muscle biopsy
Abnormal protein in muscular dystrophy and its function
Dystrophin (connects cytoskeleton of muscle fiber to surrounding extracellular matrix thru cell emmbrane)
Other than skeletal muscle, tissue commonly involved in muscular dystrophy and resultant diseases (3)
Cardiac muscle
Cardiomyopathy --> CHF and arrythmias
Elevated blood test in muscular dystrophy
Creatine kinase
Drugs for muscular dystrophy
Steroids to slow muscle degeneration
Anticonvulsants to control seizures/ muscle activity
Immunosuppressants to delay damage to muscle cells
Why do boys with muscular dystrophy have cognitive impairment?
Mutant dystrophin in neurons
Hallmark of tics associated with Tourette syndrome
Patients have insight into them, and they are suggestible
Two types of tics (other than motor vs. phonic)
Simple (blinking, limb jerking, torticollis-posturing, grunting, coughing)

Complex (head shaking, touching, hitting
When is a complex tic actually a compulsion?
If preceded by obsessive thought, anxiety, or fear
3 meds for tic suppression
Dopamine receptor antagonists (haloperidol, fluphenazine, pimozide)
Child with a nighttime seizure w/ sensorimotor manifestations on one side of the face, speech arrest, hypersalivation, and then develops a GTC
Benign rolandic epilepsy
When are anticonvulsants indicated for benign rolandic epilepsy?
Usually only if the child has experienced three or more seizures
Rx for benign rolandic epilepsy
Oxcarbazepine or gabapentin
Most common focal epilepsy in kids vs. adults
Benign rolandic epilepsy
Temporal lobe epilepsy
Infant with severe retardation, recurrent seizures, infantile spasms, hypotonia, and craniofacial dysmorphic features
Lissencephaly
2 types of lissencephaly
Miller-Dieker syndrome (epilepsy, poor feeding, and spasticity)
Isolated lissencephaly sequence (less severe)
Child w/ bouts of rapid neck flexion, arm extension, hip and knee flexion, and abdominal flexion, often with arousal from sleep
Infantile spasms
3 type of criteria for autism
Social, communication, and behavioral deficits
Autism but with normal language development
Asperger syndrome
Children with autism develop symptoms before what age?
3yo
What is very concerning in a child with developmental problems?
Prior normal achievement of developmental milestones, but now regressing (could be a progressive neurological disorder) (although children with autism may have some language regression)
Rate of epilepsy in autism
25% (vs. 1% in the general population)
Progressive onset of hearing loss, facial paralysis, headache, and imbalance
Acoustic nerve meningioma in cerebellopontine angle (CN VII and VIII are there)
Cell of origin for meingiomas
Arachnoid villi (mesoderm)
Most common tumor of cerebellopontine angle
Acoustic neuroma
Best test to elucidate etiology of unilateral sensorineural hearing loss?
MRI of internal auditory canals with gadolinium
Which primary malignancy can be diagnosed from MRI imaging of brain mets?
Malignant melanoma (hyperintense on T1 and hypointense on T2)
Steroid dose for brain tumors
10mg dexamethasone followed by 4mg q6hrs
Antibody in myositis
Anti-Jo-1
Where are the lymphocytes on muscle biopsy in polymyositis vs. dermatopyomsitis
Poly: within fascicles
Dermato: perivascular and perifascicular
Rx for inflammatory myopathies
Oral prednisone

Add MTX or azathioprine (or IV Ig) if needed)
Main association with inflammatory myopathies
Malignancy (also lung/cardiac problems in 10%)
Most common acquired myopathy in pts >50
Inclusion body myositis
Familial ALS is due to what mutation
Cu/Zn superoxide dismutase gene (chromosome 21)
What % of ALS cases are familial (AD)?
10%
Diagnostic tests for ALS
EMG/NCS

Spine imaging to r/o cervical myelopathy
Long-term carpal tunnel can cause
Thenar atrophy
Rx for carpal tunnel (1st and 2nd line)
1st line: rest, splint for immobilization, NSAIDs

2nd line: steroid or lidocaine injections, B6, PT
Surgery for carpal tunnel (what is done and what the indication is)
Severeing band of tissue

Sx for >6mo
Risk factor for brachial plexopathy
Radiation
Decreased dorsiflexion and eversion of the foot
Foot drop (peroneal palsy)
Common cause of peroneal neuropathy
Compression at fibular head
Why do partial palsies recover faster?
Local sprouting
Nerve root of peroneal nerve
L4-L5
4 types of seizures that are characterized as generalized epilepsy
Tonic-clonic
Atonic
Myoclonic
Absence
3 types of local epilepsy
Idiopathic (syndromes, genetic)
Symptomatic (trigger)
Cryptogenic
Aura of odd smell only
Simple partial seizure
4 hereditary epilepsies
Benign rolandic epilepsy
Febrile convulsions
JME
Childhood absence epilepsy
Prognosis of hereditary epilepsies
All but JME resolve spontaneously
Best AED for JME
Valproic acid
Best AED for generalized epilepsy
Valproic acid
Best AED for focal epilepsy
Phenytoin or carbamazepine
Rx for benign rolandic epilepsy
Observe unless seizures worsen
When is a febrile seizure complex (vs. simple)
Focal
>15 min
Recurs w/in 24 hours
Most common etiology of febrile seizure
HHV6
Risk of recurrence of single simple febrile seizure
30%
Rx for febrile seizures
Often intermittent prophy (rectal or oral diazepam only during febrile illnesses)
Common location of pure motor strokes
Lacunar (posterior limb of internal capsule)
Ipsilateral Horner syndrome, ataxia, and facial P&T loss; contralateral P&T loss in body; dysphagia and dysphonia
Lateral medullary syndrome
Structures contained in lateral medulla
CN V nucleus, nucleus ambiguus, lateral spinothalamic tracts, CN IX and X
What does nucleus ambiguus contain?
Motor neurons of CN IX and X
Lateral medullary syndrome is due to occlusion of?
Vertebral artery (or PICA, which is a branch of it)
Deposition in cerebral amyloid angiopathy
Beta-amyloid protein
HIV pt w/ stroke Sx and a cortical lesion on imaging
Bleeding from myocotic aneurysm
Diagnostic test for suspected bleeding from mycotic aneurysm
LP for xanthrochromia
Cause of mycotic aneurysm
Bacterial emboli from heart --> arterial wall (low virulence organism; high virulence causes meningitis or abscess)
Encephalofacial angiomatosis
Sturge-Weber (port wine stain + leptomeningeal angiomas)
Broca's aphasia cause
Left MCA infarction
Pts with bleeds <40yo likely have
AVMs (aneurysms don't bleed til later)
Aneurysm where can compress CN III?
Posterior communicating artery
Cause of transient monocular blindness in carotid artery disease?
Embolism to central retinal artery
Gaze deviation indicates
Ipsilateral frontal lobe damage
Where are expressive vs. receptive language centers?
Expressive: frontal
Receptive: temporal
Where do the language centers communicate?
Arcuate fasciculus
Problem with repetition is a
Conduction aphasia
Speech limited to repetition implies
Mixed transcortical aphasia
Tunnel vision
Conversion disorder or malingering
Concentric constriction (visual field is overall smaller but enlarges as test screen moves away)
Neurosyphilis
Lack of red reflex
Congenital cataracts
Presence of white reflex
Scar or retinoblastoma
Sturge-Weber has a risk of what eye problem?
Glaucoma
Rx for CMV
Ganciclovir
Acute central scotoma
Methyl alcohol intoxication
How to differentiate pappillitis and papilledema?
Former has visual loss
Rx for pseudotumor in pregnant woman
Repeated LPs
Cause of APD
Optic atrophy from optic neuritis
Retinal exam changes from HTN
Segmental narrowing of arterioles and nicking
Retinal microaneurysms usually indicate?
DM
Most common ocular motor paresis is of
CN VI
CN VI nucleus is where?
Pons
Which CN is most commonly injured w/ facial trauma?
CN IV (superior oblique) b/c extends far into orbit
What do pts develop to compensate from CN IV loss?
Head tilt
Which CNs are affected by herpes zoster ophthalmicus?
CN V ad IV
Diabetic injury to CN III usually spares?
Pupillary constriciton (whereas that goes first with compression)
INO indicates
Mesencephalic or pontine injury
Rapid downward deviation of both eyes followed by slow upward conjugate eye movements
Ocular bobbing, usually from pontine damage
Nystagmus while locking at a rotating drum w/ stripes
Optokinetic nystagmus (normal)
Which drugs cause a syndrome that can resemble hypertensive encephalopathy?
Cyclosporine and tacrolimus (reversible posterior leukoencephalopathy)
Inferior visual field defect
Ischemic optic neuropathy
Appearance of ischemic optic neuropathy on retinal exam (early and late)
Initially swollen optic nerve, then pale optic nerve
Lantern jaw, prominent nose, spade shaped hands, DM and menstrual problems
Pituitary problem, e.g. acromegaly
Temporal lobe damage eye findings
Superior homonymous quadrantonopsia