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Neuro Flashcards

1
Q

Phenytoin Mechanism, Kinetics, AE/Toxicity

A

Mechanism: Na channel inactivation
Kinetics: zero order kinetics

Most common: gingival hyperplasia (gum hypertrophy)

During infusion: hypotension, febrile reaction, bradycardia, arrhythmia

Neurologic AE: ataxia, choreoathetoid movement, dysarthria, nystagmus, diplopia, peripheral neuropathy

Other: Purple glove syndrome, TEN, osteomalacia, hypothyroidism

Fosphenytoin has decreased AE, also an IM route

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2
Q

MS Disease Modifying Therapy

A

Corticosteroids - tx acute attacks

Glatiramer - decreases flares
Interferon beta-1a - decreases relapses, lesions, and rate of disability
Interferon Beta-1B - decreases flares, lesions, rate of disabilty

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3
Q

How common is MS?

A

1 in 1000 in the US

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4
Q

Canavan disease

A

Spongiform demyelination, defect in aspartoacylase on Ch 17, AR
Macrocephaly

Developmental regression at 3-6 month, with sx including posturing, rigidity, myoclonus.

Lab: N-acetyl-L-aspartic acid in blood, urine, brain

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5
Q

What drugs treat spasm symptoms in MS?

A

Baclofen
Tizanidine
Benzodiazepines

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6
Q

Uhthoff phenomenon

A

Symptoms of MS increase in the heat (shower, summer)

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7
Q

Marchiafava-Bignami disease

A

Demyelination of corpus callosum

Seen in chronic alcoholism

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8
Q

ADEM

A

Demyelinating disease, rapid, often post-infectious, often fatal.

Damage to small blood vessels and perivascular tissues.

MRI/CT: rapidly evolving white matter damage
high ESR
CSF with high pressure, elevated protein/RBC/WBC, nl glucose

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9
Q

Pelizaeus-Merzbacher disease

A

Sudanophilic leukodystrophy.

X-linked

Pendylar nyastagmus
tremor w/o seizure, optic atrophy, choreoathetotic limb movemets, seizures, gait ataxia

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10
Q

Leber optic atrophy

A

Presents with centrocecal scotoma (enlargement of physiologic blind spot to impinge on central vision), painless, bilateral or sequential

Hereditary, due to mitochondrial DNA mutation, mainly in men

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11
Q

Alexander disease

A

Leukodystrophy of glial fibrillary acidic protein
Ch 11 or 17, AR

Childhood onset of macrocephaly, seizures, spasticity, developmental delay

Abnormal protein deposits: Rosenthal fibers in astroglial cells

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12
Q

Adrenoleukodystrophy

A

Progressive degenerative disease of white matter and cerebellum.
X-linked

Sx limb ataxia, nystagmus, mental retardation, adrenal dysfunction; may be neuropathy or myelopathy in adults

Lab: low serum cortisol
Defect in VLCFA oxication

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13
Q

Vasogenic vs Cytotoxic Edema

A

Vasogenic: Leakage of fluid from capillaries, disruption of BBB, mainly affecting white matter. Seen with tumors, abscesses, maturing contusion/hemorrhage. MRI shows hyperintensity. Tx with steroids.

Cytotoxic: Extracellular water goes into cells and causes swelling. No change in capillary permeability or disruption of BBB. Mostly affects grey matter. Seen with cerebral ischemia. Diffusion weighted imaging shows decrease/restricted diffusion.

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14
Q

Causes of brain hemorrhage

A

Most common: hypertension, trauma, iatrogenic (blood thinners)

Elderly: cerebral amyloid angiopathy
Children: vascular malformations

Other: aneurysm, vasculitis, tumor, infection, septic emboli

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15
Q

Drug-induced optic neuritis

A

Ethambutol, in tx of tuberculosis

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16
Q

Infectious causes of optic neuritis

A

Lyme, Syphilis, HIV, EBV, CMV, TB, toxoplasmosis, toxocariasis, Bartonella

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17
Q

Typical optic neuritis

A

Young, white adult, unilateral sx.

Mild periocular pain worse on eye movement, moderate uniocular vision loss with spontaneous improvement, nl or swollen optic disk. May be worse with heat.

30-70% are first presentation of MS.

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18
Q

Carbidopa/Levodopa

A

Given with

AE: Low blood pressure, worsen hallucinations,

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19
Q

Entacopone

A

COMT Inhibitors

AE: diarrhea

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20
Q

Rasagiline

A

MAO-B inhibitor

Neuroprotective (decrease progression of parkinson’s disease)

AE: well tolerated

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21
Q

Dopamine agonists

A

Pramipexole (Mirapex)
Ropinerole (Requip)

AE: impulse control behaviors (e.g. gambling, spending, eating)

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22
Q

Rytary

A

Extended release Carbidopa/Levodopa

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23
Q

Migraine HA, sx and tx

A

Unilateral, throbbing/pulsating, 3-72 hours, with photophobia, phonophobia, nausea, exacerbated by movement.

Classic = with aura (preceding, during, or following HA); common = without aura

Abortive tx: sumatriptan (oral, subq, nasal, sublingual), ergots, caffine, metoclopramide, prochlorperazine, promethazine

Prophylactic tx: propranolol, amytriptyline, valproate, verapimil

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24
Q

Cluster HA

A

Subset of TAC

Unilateral, trigeminal pain (usually V1), ipsilateral autonomic sx (lactimation, injection, edema, rhinorrhea, Horner’s).

Excrutiating pain, orbital/temporal. May occur over weeks-months with high frequency

Abortive tx: high flow O2, triptan
Steroids may shorten duration/reduce frequency
Avoid alcohol
Prophylaxis: verapimil, lithium

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25
Q

Trigeminal neuralgia

A

Neuropathic pain, severe, in V2 or V3. Short episodes of electrical-like sensation, may be triggered by tactile sensation. Pain is lancinating, paroxysmal (worse with cold), unilateral

Tx carbamazepine (1st line), phenytoin, gabapentin

Associated with MS

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26
Q

Basilar migraine

A

Women>men
Aura preceding headache. Aura may have visual changes, irritability, psychosis, stupor, syncope, coma. Aura lasts 20-30 min.

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27
Q

What is the physical exam finding of tension headache?

A

Reduced neck ROM and paracervical tenderness

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28
Q

Sumatriptan: mechanism, use, toxicity

A

5-HT 1b/1D agonist. Inhibits trigeminal nerve activation, induces vasoconstriction, prevents vasoactive peptide release

Tx acute migraine or cluster headaches (abortive)

Contraindicated in CAD or prinzmetal angina due to vasospasm

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29
Q

What medication do you use to treat VZV zoster?

A

Oral acyclovir.

IV acyclovir used in meningitis, but it has renal toxicity so must be given with saline and limited in CKD, diabetic nephropathy.

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30
Q

Schwannoma

A

Common in middle-aged women, around vestibular (VIII), involving trigeminal and facial nerves at cerebellopontine angle

Bilateral are associated with neurofibromatosis 2 (plus juvenile cataract, meningioma, and ependymoma)

Tx with stereotactic radiosurgery, esp if

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31
Q

Metabolic causes of seizure

A 
Hypoglycemia
Nonketotic hyperglycemia - focal motor seizures
Hyponatremia
Hypernatremia
Hypocalcemia (most often in neonates)
Hypomagnesemia
Renal failure / uremia
Hyperthyroidism (exacerbate epilepsy)
Acute intermittent porphyria
Alcohol and benzodiazepine withdrawl
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32
Q

What are four neurologic manifestations of RA?

Hint: two structural, one 2/2 drug, one associated dz

A

Atlantoaxial subluxation –> cervical myelopathy
Compression or entrapment neuropathy –> carpal tunnel
PML (increased risk in patients treated with rituximab.
Hyperviscosity (APS)

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33
Q

Blowout fracture: dx and complication

A

Trauma, limitation of upward gaze, Increased intraocular pressure

Complication: entrapment of inferior rectus muscle –> limitation of upward gaze

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34
Q

Cavernous sinus

What cranial nerves pass through the cavernous sinus?

A

CN III, IV, V1, V2 in the lateral wall

VI runs through it, next to the internal carotid

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35
Q

Alcohol withdrawal

A

Seizure day 1
DT day 2-4 (confusion, arrhythmia, autonomic hyperactivity)

Treat with benzodiazepines

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36
Q

Carpal tunnel syndrome

A

Compression of median nerve

Sx: weakness of thenar muscles, esp Abductor Pollicis Brevis decreased sensation of 3.5 digits, Tinel’s sign, Phalen’s sign. Numbness may awaken patient from sleep.

Nerve conduction studies: impaired

Tx: nocturnal wrist splinting, surgical decompression, steroid injections, oral steroids

From C5-T1

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37
Q

Celecoxib

A

COX2 selective NSAID

used to tx osteoarthritis, RA, ankylosing spondylitis

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38
Q

glucosamine and chrondroitin

A

Supplements used to treat OA

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39
Q

Interferon beta-1a: side effects

A

AE: flu-like, anemia, depression, development of neutralizing antibodies

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40
Q

Interferon beta-1b: side effects

A

Flu-like sx, depression, development of neutralizing antibodies

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41
Q

Glatiramer acetate side effects

A

AE: injection rxn, injection-related chest pain, SOB

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42
Q

Natalizumab: mechanism, AE

A

Monoclonal antibody against alpha-4-integrins

Admin in monthly infusions

AE: PML, hepatotoxicity

Do not use in combination with other agents

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43
Q

Fingolimod: mechanism, AE

A

Mixed agonist/antagonist of sphingosine-1P1 receptor.
First oral MS medication.

AE: bradycardia, leukopenia
Monitor with ECG during first administration

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44
Q

Botulinum

A

Prevents release of stimulatory acetylcholine

Leads to flaccid paralysis
Descending paralysis, dilated pupils

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45
Q

Neuroleptic malignant syndrome

A

Muscle rigidity, fever, autonomic instability, delirium, elevated CK, elevated WBC

2/2 to sudden reduction in dopamine activity (blockade of dopamine receptors aka antipsychotics, withdrawal of dopaminergic agents)

Tx: dantrolene (inhibit Ca++ ions, tx rigidity), bromocriptine (dopamine agonist)

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46
Q

Serotonin syndrome

A

Elevated body temp, agitation, hyperreflexia, tremor/myoclonas, diarrhea, sweating, dilated pupils, seizures. May have high CK

2/2 use of SSRI, MAOI, TCA, meperidine, dextromorphan (cough syrup), trazadone, mitrazapine, tramadol,

Tx benzos, cyproheptadine (serotonin antagonist)

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47
Q

Craniopharyngioma

A

Suprasellar tumor, slow growing but invasive.

Calcifications on imaging

Associated with endocrine dysfunction (central DI, short stature)

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48
Q

What are some types of dopamine antagonists?

A

Atypical antipsyhotics (clozapine, olanzapine, quetiapine, risperidone)

Antiemetics (metoclopramide, droperidol)

TCAs (amoxapine, trimipramine, clomipramine)

All can cause drug-induced parkinsonism

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49
Q

What is citalopram?

A

SSRI

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50
Q

Narcolepsy

A

loss of orexin NT
associated with cataplexy (collapse with laughter or strong emotion)

tx with stimulants during day (e.g. modafinil, dextroamphetamine), benzos at night

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51
Q

What do you use to treat restless leg syndrome?

A

Pramipexole (dopamine agonist)

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52
Q

Lenox-Gastaut syndrome

A

Pediatric disorder by age 8 - mental dysfunction, multiple seizure types, 1-2 Hz generalized waves on EEG (slow spike and wave)

Associated with h/o infantile spasms (West syndrome)

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53
Q

Landau-Kleffner syndrome

A

Loss of language with abnormal EEG in sleep

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54
Q

What part of the brain do olfactory aura/hallucinations come from?

A

Mesial temporal lobe, or hippocampus/parahippocampus

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55
Q

What is West Syndrome, and what is the best treatment?

A

West syndrome: triad of infantile spasm, neurologic or psychomotor deterioration (loss of milestones), inter-ictal EEG pattern of hypsarrhythmia (chaotic, high amplitude with multifocal spike and slow wave discharge)

Sx stereotyped clusters and axial contractions, often when infant awakens, between 3 mo and 1 year

Tx ACTH

May be associated with tuberous sclerosis (with hypopigmented spots)

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56
Q

What is the best treatment for absence seizures?

A

Ethosuximide or valproate

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57
Q

What is the best tx for complex partial seizures? And what are common AE?

A

Leviteracetam

AE = neuropsychiatric symptoms including irritability, agitation, depression

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58
Q

Frontal lobe seizure

A

Dramatic, prominent motor manifestations, often nocturnal lasting 15-45 seconds.

May have loud vocalizations, Jacksonian march

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59
Q

Temporal lobe seizure

A

Aura (epigastric rising sensation, smell, sense of fear via amygdala), automatisms, dystonic posturing
post-ictal fatigue, confusion

ictal EEG shows discharge best developed in temporal lobe

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60
Q

Occipital lobe seizures

A

Sudden visional changes, visual halucinations

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61
Q

Mesial temporal lobe epilepsy

A

Loss of hippocampal neurons and gliosis in CA1

Partial seizures, with autonomic and psychic aura, localized repetitive clonic activity (epilepsia partialis continua)

Caused by stroke, tumor, rasmussen encephalitis (pediatrics)

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62
Q

Childhood absence epilepsy

A

Onset 4-8yo, 45% with family history
EEG 3Hz spike wave, elicited with hyperventilation
“staring spells”

Tx ethosuxamide, depakote, lamtrogine

Generally outgrow seizures

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63
Q

Juvenile Myoclonic epilepsy

A

Onset 13-18yo, clonic movements involving arms and shoulders symmetrically

Tx depakote, lamotrigine, topiramate

Generally no remission of seizures, need AED for life

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64
Q

Psychogenic seizure

A

Limb movement discordantly/alternating, speaking or maintained mentation with generalized body movements, hip thrusting

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65
Q

Benign Rolandic epilepsy of childhood

A

4-8yo, family history of febrile seizures or epilepsy

Unilateral paresthesias on face, unilateral clonic activity in face –> drooling, speech impairment, may secondarily generalize. Occur shortly after child falls asleep

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66
Q

Febrile seizures

A

3mo to 3 years
3% chance of developing epilepsy downstream
May have only mildly elevated temp at time of seizure

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67
Q

Which antiepileptics should probably not be used in pregnancy and why?

A

Phenytoin - fetal hydantoin syndrome (IUGR, dysmorphic facial features, hypoplastic nails and distal phalanges)

Valproate and carbamazepine - neural tube defects

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68
Q

What neuroanatomy may cause coma?

A

Reticular activating system (diffuse brainstem), bilateral thalami, bilateral cortex

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69
Q

Corneal reflex (afferent and efferent, anatomy)

A

AFFERENT: CN V
EFFERET: CN VII
Assesses pons

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70
Q

Gag reflex (afferent and efferent, anatomy)

A

Afferent: CN IX
Efferent: CN X
Assesses medulla

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71
Q

Persistent vegetative state

A

Regained elements of wakefulness, not awareness, >4 weeks

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72
Q

What drug is used to treat Lennox-Gaustat syndrome, and what is the potential adverse effect?

A

Felbamate

Aplastic anemia, liver failure

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73
Q

How do you manage acute back pain?

A

Maintain moderate activity, use NSAIDS or acetaminophen
Consider muscle relaxants, spinal manipulation, or brief course of opioids

Acute defined as 4-6 weeks

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74
Q

General paresis

A

Manifestation of neurosyphilis

Dementia, delusion, dysarthria, tremor, seizures, spasticity, Argyll Robertson pupils

Monocytic pleocytosis, +VDRL

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75
Q

Rett syndrome

A

X linked disorder
Young girls, rapid regression in 2nd year of life, with characteristic repetitive hand movements (wringing), hypotonia, gait abnormalities, seizures

76
Q

Transcortical sensory aphasia

A

Reduction in ability to understand complex linguistic structures

Seen in alzheimers

77
Q

Whipple’s disease

A

GI complaints + seizures, myoclonus, ataxia, dementia

Pathognomonic: oculomasticatory myorhythmia (pendular convergence movements of eyes with contractions of masticatory muscles)

Biopsy of jejunum shows PAS positive cells

Tx antibiotics

78
Q

Sensory deficit or pain in anterior thigh

A

Femoral nerve

Leg flexion at hip, leg extension at knee
Quadriceps weakness, diminished patellar reflex.
Won’t lose adduction - if lose adduction, think L3 root

79
Q

Sensory deficit in medial thigh

A

Obturator nerve

Adduction of thigh

80
Q

posteror-lateral leg, plantar foot sensory deficit

A

Posterior-lateral leg = sural
Plantar foot = Tibial

Flexion of the knee, plantar flexion of foot
S1 nerve root

81
Q

Sensory deficit in lateral leg, dorsal foot

A

Superficial peroneal nerve

Foot eversion

82
Q

Dorsal foot around big/2nd toe sensory deficit

A

Deep peroneal nerve
L4-L5
Foot dorsiflexion, toe extension

83
Q

Carbon monoxide poisoning

A

Sx red skin, confusion, headache

H/o smoke inhalation, automobile exhaust

Dx serum carboxyhemoglobin level

CT: Low attenuation in globus pallidus
MRI findings: high signal in T2 and flair in globus pallidus

Tx 100% O2, hyperbaric O2

84
Q

Ecstasy and seizure

A

Ecstasy induces SIADH –> Hyponatremia –> cerebral edema, seizure –> herniation
May be febrile, high CK, HTN
H/o partying, drinking lots of water

85
Q

Spinal symptoms in a person with local extremity infection?

A

If immunosuppressed or risk factor, possibly spinal abscess.

86
Q

What is used to treat sleep phase disorder?

A

Melatonin or phototherapy

87
Q

Lithium toxicity

A

Drowsy, hyperreflexia, confused, leukocytosis, course tremor, tinnitus, polyuria

Tx discontinue lithium, hemodialysis

88
Q

Acute treatment for GCA

A

Corticosteroid therapy

89
Q

Neurofibromatosis Type 1

A

AD, ch17
Benign skin tumors, learning disability, cafe au lait spots, plexiform neurofibromas (incl schwannoma), Lisch nodules (spots in colored part of iris), associated with optic glioma, freckles in axilla or inguinal (Crowe’s sign)

90
Q

Neurofibromatosis Type 2

A 
AD, 22q12
Bilateral schwannoma (acoustic neuroma), ependymoma, meningioma, juvenile cataract, glioma
91
Q

ethylene glycol

A

Sweet odor, crystals in urine, anion gap acidosis

92
Q

CJD

A

Myoclonus, dementia
14-3-3 protein in CSF
Imaging: T2/Flair hyperintensity on basal ganglia, thalamus

93
Q

Syringomyelia

A

C8-T1
Cystic = hydromyelia
Loss of pain/temperature first, progress to weakness, bilateral
seen with Chiari 1 malformations

94
Q

Tabes dorsalis

A

Progressive demyelination of dorsal columns

Later sensory ataxia

95
Q

What is the treatment for GBS?

What infections most likely cause GBS?

A

IVIG, plasmapheresis

Campylobacter and Mycoplasma
CMV, EBV

96
Q

What drugs are used to treat alzheimers?

A

Acetylcholinesterase inhibitors
Donezepil
Rivastigmine
Galantamine

After that, Memantine (NMDA receptor antagonist)

97
Q

BPPV Tests

A

Dix-Hallpike - to diagnose
Epley Maneuver - to fix
Mechanism: loose otolith

98
Q

Four causes of optic chiasm compression?

A

Pituitary adenoma, craniopharyngioma, meningioma, aneurysm at the anterior communicating artery

99
Q

How do you treat cauda equina secondary to tumor invasion of the epidural space?

A

Immediate corticosteroids

Followed by surgery or radiation therapy

100
Q

What class of drug do you use to treat acute dystonia?

A

Anticholinergic

101
Q

Ciguatoxin

A

Dinoflagellates consumed by reef fish
Acts on voltage-gated Na channels, increasing permeability

Sx abd pain, n/v/d, parasthesia, headache, fatigue, myalgia, temperature reversal (cold objects feel hot)

102
Q

Lathyrism

A

Slow onset spastic paraperesis

Excess of chickling pea, in famine,

103
Q

Tick paralysis

A

holocyclotoxin, acts at presynaptic release of neuromuscular junction

no elevated spinal fluid protein (compared to GBS)

remove tick and dramatic improvement

104
Q

Manganese poisoning

A

Inhalation
Sx parkinsonism, axial rigidity, dystonia
Neuronal loss in globus pallidus, putamen, caudate

105
Q

Ergot poisoning

A

associated with rye fungus Claviceps purpurea
Vasoconstricting agent

Degeneration of posterior column, peripheral neuropathy

106
Q

Mercury poisoning

A

Personality changes, tremor, ataxia

Used in paper, pulp, electrochemical manufacturing

107
Q

Lead poisoning

A

Bilateral neuropathy, esp at radial nerve (wrist drop, finger drop)
abdominal pain, constipation, anemia, basophilic stippling of erythrocyte precursors, linear discoloration of gingival margin
May cause ataxia and tremor in chidlren exposed to low levels, chronic exposure leads to impaired psychomotor dev, retardation, brain edema (with herniation risk)

tx penicillamine

108
Q

Arsenic poisoning

A

Sc encephalopathy, tonic-clonic seizures, peripheral neuropathy, hemolysis/anemia, white lines transverse across fingernails

109
Q

Tay Sachs

A

Hexosaminidase A deficiency
Ch 15, AR
Disease of ganglioside storage

Sx cherry macula, macrocephaly, retardation, seizures, blindness

110
Q

Gaucher disease

A

beta-glucosidase deficiency
Ch 11, AR

hepatomegaly, splenomegaly, low platelets, anemia

May have acute infantile form, causing spasticity, bulbar weakness, fatal by ~3yo

111
Q

Neurologic complications of end-stage renal disease

A

Peripheral neuropathy – tx with dialysis

Restless leg syndrome – tx with doapminergic agonist or l dopa

112
Q

What is the visual symptom of tobacco/alcohol abuse?

A

Enlarged blind spot / amylopia

113
Q

Pellagra

A

Nicotinic acid deficiency

Irritability, memory loss, dermatitis, anemia

114
Q

Vitamin E deficiency

A

Ataxia / spinocerebellar degeneration, polyneuropathy, pigementary retinopathy
High CK, high bilirubin 22/2 cholestatic hepatobiliary disorder

115
Q

What four drugs can be used to treat essential tremor?

A

Propranolol, primidone, topimax, gabapentin

116
Q

Wernicke’s encephalopathy classic triad

A

Confusion, ataxia, opthalmoplegia

Tx with thiamine, then glucose

117
Q

Criteria for depression (9)

A 
Appetite or changes in weight
Sleep disturbances
Anhedonia
Depressed mood / irritability
Changes in energy
Changes in concentration or executive function
Guilt or worthlessness
Suicidal ideation
Psychomotor retardation
118
Q

Parsonage-Turner Syndrome

A

Acute brachial neuropathy
May be post-infectious
Arm pain, weakness, numbness, atrophy, may be unilateral

119
Q

Hypokalemic periodic paralysis

A

Autosomal dominant channelopathy
Muscle weakness or paralysis with fall in K levels
Adolescence
Occur on awakening of after sleep, associated with high carb or high sodium meals, sudden temperature changes, noise, flashing lights

120
Q

Hemiballismus - where is the lesion?

A

Subthalamic nucleus

121
Q

Status epilepticus management

A

1) diazepam IV push
2) fosphenytoin or valproate IV push
3) refractory, with EEG monitoring –> infusion propofol, midazolam, or phenobarbitol

122
Q

Anti-emetics that are dopamine antagonists

A 
Domperidone
Metoclopramide
Alizapride
Prochlorperazine
Promethazine
Trimethobenzamide
123
Q

What anti-emetic does not affect dopamine?

A

Odansetron. It is a serotonin antagonist.

124
Q

What disease is associated with predominantly medial temporal lobe atrophy on MRI?

A

Alzheimers vs MCI (more atrophy = alzheimer’s dementia)

125
Q

What disease is associated with predominant occipital hypometabolism on PET?

A

Lewy Body Dementia

126
Q

What is the most common cause of cerebellar hemorrhage? And what are the symptoms?

A

Hypertension

Sx nausea, vomiting, acute onset of occipital headache, , truncal ataxia (inability to walk), dizziness, vertigo

127
Q

Myesthenia Gravis tx

A

1st - acetylcholinesterase inhibitors (pyridostigmine)

If remain symptomatic, then go to immunosuppression (steroids, azathioprine, mycophenalate mofetil)

128
Q

Infant with failure to thrive, bilateral cataract, jaundice, hypoglycemia – what does he have?

A

Galactose-1-phosphate uridyl transferase deficiency (galactosemia)

Tx by eliminating galactose from the diet

129
Q

What neurological reaction can metoclopramide induce?

A

Dystonia

or tardive dyskinesia, or parkinsonism

130
Q

What tumors most often metastasize to the brain?

A

Lung > Breast > unknown primary > melanoma > colon

If multiple mets: lung cancer, malignant melanoma
If solitary met: breast, colon, renal cell carcinoma
If it’s easily bleeding: melanoma, renal cell, choriocarcinoma

131
Q

Lhermitte’s sign

A

Increased “electrical” sensation down spine or limbs with flexion of the neck. Suggests MS.

132
Q

Paraneoplastic cerebellar syndrome - what antibody is associated?

A

Anti-Yo, purkinje cell cytoplasmic antibody Type 1

133
Q

Alcohol abuse is associated with what damage in the cerebellum?

A

Vermal midline cerebellar ataxia

134
Q

What antibodies are associated with small cell lung cancer?

A

Anti-Hu

As well as Lambert-Eaton syndrome

135
Q

What antibody is associated with seronegative myesthenia gravis?

A

Anti-MUSK

136
Q

What antibody is associated with polymyositis?

A

Anti-Jo

137
Q

Tourette’s

A

Motor and verbal tics, with no tic-free period for 3 months
Comorbid with OCD, ADD, depression

Tics peak just before puberty, usually resolve after puberty. Comorbid anxiety, OCD, ADD do not improve.

tx tics with alpha-2 antagonists (clonidine)

138
Q

What post-infectious condition is associated with VZV?

A

Acute postinfectious cerebellar ataxia

25% have had chicken pox, 80% have had some preceding infection

139
Q

Meralgia paresthetica

A

burning sensation and loss of sensation over anterolateral thigh. Comes from entrapment of lateral femoral cutaneous nerve near inguinal ligament. Tender palpation of inguinal ligament.

140
Q

Compression of ulnar nerve

A

parasthesia in pain in 5th/4th digits, weakness in dorsal interossei and abductor digiti minimi. From compression at elbow.

From C8-T1

141
Q

Radial nerve entrapment

A

Wrist drop, sensory loss on dorsum of hand. Weakness of triceps, brachioradialis, supinator, and wrist/finger extensors.

Compression at axilla, spiral groove or forearm

From C5-T1

142
Q

Name the reflexes and their nerve roots (use the song)

A

Achilles - S1/2
Patellar - L3/4
Biceps - C5/C6
Triceps - C7/8

Cremaster - L1/L2
Anal wink - S3/S4

143
Q

What feature differentiates loss of C5 vs C6?

A

In both, lose or impair biceps reflex.

In C5, sensory loss and weakness of deltoid and supraspinatus
In C6, sensory loss extends to thumb, weakness of biceps and brachioradiatlis

144
Q

What differentiates loss of L3 and L4?

A

Both lose patellar reflex

L3 - medial thigh sensory loss, adduction (differentiate from femoral nerve) loss and quadriceps

L4 - medial leg sensory loss, quadriceps and anterior tibial weakness

145
Q

What does triceps weakness indicate?

A

C7 nerve root syndrome

146
Q

What does deltoid weakness indicate?

A

C5 nerve root syndrome or axillary nerve

147
Q

Gerstmann Syndrome

A

AFAR: Agraphia, Finger agnosia, Acalculia, Right-left confusion

Due to MCA stroke damaging dominant parietal lobe

148
Q

MCA stroke

A

Contralateral motor and sensory deficit of upper limb and face

If dominant hemisphere - aphasia
If nondominant hemisphere - hemineglect

149
Q

Lacunar stroke

A

Hypertension + small penetrating arteries
May affect cerebellum, pons, internal capsule/basal ganglia, thalamus

Most frequent sx: mute motor hemiparesis (posterior internal capsule) also pure sensory, dysarthria-clumsy hand (pons or internal capsule), and ataxic hemiparesis (basis pontis, ipsilateral motor and cerebellar sx)

150
Q

ACA stroke

A

Contralateral lower limb paralysis and loss of sensation

151
Q

Medial medullary syndrome

A

Contralateral hemiparesis, decreased contralateral proprioception, tongue deviates ipsilaterally

Artery: ASA

152
Q

Lateral Medullary Syndrome

A

Artery: PICA

Vomiting, vertigo, nystagmus, ataxia, dysmetria
Dysphagia, hoarseness
Loss of little three in ipsilateral face and contralateral body
Ipsilateral horner’s syndrome

153
Q

Lateral pontine syndrome

A

Paralysis of face, decreased taste, lacrimation, salivation
Loss of little three in ipsilateral face and contralateral body

Artery: AICA

154
Q

Locked in syndrome

A

Basilar artery stroke

Preserved consciousness and blinking, quadriplegia, loss of voluntary facial/mouth/tongue movements

155
Q

Weber syndrome

A

PCA / base of mibrain

Contralateral Weakness – upper and lower extremity Corticospinal tract
Ipsilateral Lateral gaze weakness CN 3

156
Q

Thalamic pain syndrome

A

PCA to thalamus
Contralateral Hemisensory loss – all modalities
Contralateral Hemi-body pain

157
Q

Cortical blindness

A

PCA / bilateral occipital
Visual loss – bilateral
Unawareness or denial of blindness

158
Q

Homocystinuria

A

Marfan habitus (tall, pectus deformity, skin and joint hyperelasticity)

Cerebrovascular event (thrombus)
Intellectual disability
Fair hair / eyes, eye problems

159
Q

Where is broca’s area?

A

Inferior frontal gyrus, fed by MCA

160
Q

Where is wernicke’s area?

A

Superior temporal gyrus, MCA

161
Q

What do the Rinne Test and Weber Test say?

A

Weber test - tuning fork to forehead. If sound is heard better to the right: conductive hearing loss on the right or sensorineural loss on the left

Rinne - tuning fork to mastoid process then air. If sound still heard, normal. If cannot be heard, bone conduction > air conduction, and that ear has conductive hearing loss.

162
Q

What are types of ring-enhancing lesions?

A

Brain abscess (toxoplasmosis if CD4

163
Q

Meperidine

A

Anticholinergic AND Analgesic

164
Q

Von Hippel Lindau syndrome - what inheritance pattern is it, and what are four commonly associated tumors?

A 
Autosomal dominant, ch3
Cerebellar hemangioblastomas (resect ASAP, they bleed)
Renal cell carcinoma
Angiomatosis
Pheochromocytoma

Plus hepatosplenomegaly, cerebellar dysfunction
May have cafe au lait spots

165
Q

Fragile X Syndrome

A

Elongated face, protruding ears, large testicles, stereotypic movements (hand flapping), intellectual disability, social anxiety, hypotonia
Inheritance: x linked dominant
Men: hyperextensible joints, prominent thumbs
Women: mild retardation in half

166
Q

Metachromatic leukodystrophy

A

Autosomal recessive, deficiency of arylsulfatase A
Ch 22, AR
Late infant: Spasticity, ataxia, blindness, seizures, coma
Pediatric: impaired school, dementia –> spasticity, seizures, coma
May also have cherry red spot
Adult: schizophrenia, psychosis
Nerve conduction shows slowed motor and sensory
Biopsy of nerve shows sulfatide in Schwann cells or positive urine sulfatides

167
Q

What is hartnup disease, and what is the therapy?

A

Malabsorption of tryptophan
Sx red scaly rash, episodic cerebellar ataxia, dev delay, emotional lability
Tx Nicotinamide

168
Q

Sturge Weber Syndrome

A

Port-wine stain in V1 distribution, glaucoma, seizures, retardation, leptomeningeal angioma
Calcifications in cortex in “railroad track” pattern reflecting calcified subependymal glial nodules

169
Q

Tuberous sclerosis - what are the characteristic features, cutaneous findings, and CNS tumors?

A

Autosomal dominant
TSC1 on ch9, TSC2 on ch16

Hamartia (cortical tubers)
Hamartomas (subependymal nodules, facial angiofibroma/adenoma sebaceum)

CNS: cortical tubers, subependymal nodules, giant cell astrocytoma
Cutaneous: adenoma sebaceum, ash-leaf spots, shagreen patches

Also associated with retinal phakomas (astrocytic hamartomas), 65% have mental retardation, also cardiac rhabdomyoma or angiomyolipoma of kidneys

170
Q

Niemann-Pick Disease

A

Sphingomyelinase defiency
Ch 11, AR
Cherry red spot
Self-mutilation

171
Q

Krabbe disease

A

Defect in galactosylceramide P-galactosidase
Ch 14, AR

Positive PAS (periodic-acid Schiff) granules in globoid cells

172
Q

Hurler syndrome

A

Defect in a-L-iduronidase
Ch 4, AR

Clouding of cornea, facies, dwarfism

173
Q

Hunter’s syndrome

A

Defect in iduronate sulfatase
X-linked
Facies, dwarfism, no corneal clouding

174
Q

Leigh syndrome

A

Mitochondiral defect
X linked or AR
MRI shows bilateral putamen hyperintensity

175
Q

Rett’s syndrome

A

Defect in methyl-CpG-biding protein 2
X linked
Exclusively occurs in girls
Microcephaly, autism, hand-wringing

176
Q

Ataxia telangiectasia

A

Ch 11, AR
Truncal ataxia, progressive dementia, telangiectasia
Susceptibility to infeciton, high leukemia/lymphoma rates

177
Q

Down syndrome

A

Trisomy 21

upslanting palpebral fissues, protruding tongue, simian crease, brushfield spots

178
Q

Prader-Willi

A

Paternal ch15

Mental retardation, emotional lability, insatiable appetite

179
Q

Angelman’s syndrome

A

Maternal ch15

Mental retardation, inappropriate happy behavior, seizures

180
Q

What is empiric therapy for bacterial meningitis?

A

Ceftriaxone and vancomycin, + ampicillin for elderly

Possibly just ampicillin for infants

181
Q

What are the common causes of bacterial meningitis?

A

Newborns: group B strep, listeria, e. coli, strep pneumo
Adults: N meningitidis, strep pneumo
Elderly: N meningitidis, strep pneumo, listeria, HiB, group B strep

182
Q

Wernicke encephalopathy

A

Thiamine deficiency

Triad of ataxia, confusion, ophthalmoplegia, developing over days-weeks

Progresses to Korsakoff’s syndrome, with anterograde amnesia and confabulation

183
Q

Subacute combined degeneration

A

B12 deficiency
Disease in dorsal columns and lateral corticospinal tract

Parasthesia in hand/feet, weakness and spasticity, macrocytic anemia.

Elevated homocystine and MMA

184
Q

How do you differentiate steroid-induce myopathy, PMR, statin induced myopathy nad inflammatory myopathy?

A

Steroid-induced: normal ESR and CK, less pain
PMR: pain and stiffness in shoulder and pelvic girdle, high ESR but normal CK. Responds to steroids quickly.
Statin: Normal ESR, high CK, prominently pain/tenderness
Inflammatory: high ESR and high CK, may have skin rash/arthritis

185
Q

Tabes dorsalis

A

Sensory ataxia
Lancinating pains
Argyll-Robertson pupil (accomodates but does not respond to light)

186
Q

Medial pontine syndrome

A

contralateral ataxia of face, trunk, and lims

Shelf Exams (2 decks)

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