Neuro X Flashcards

1
Q

deficient enzyme in Krabbe disease

A

galactocerbosidase

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2
Q

what builds up in krabbe’s?

A

1) galactocebroside

2) psychosine

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3
Q

Krabbe pathophys

A

galactocerboside + psychosine destroy myelin sheath.

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4
Q

findings in Krabbe’s

A

1) peripheral neuropathy
2) developmental delay
3) optic atrophy
4) globoid cells

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5
Q

Metachromatic leukodystrophy pathophys

A

Buildup of sulfatides leads to impaired production and destruction of myelin sheath.

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6
Q

Findings in metachromatic leukodystrophy

A

1) central and peripheral demyelination with ataxia and dementia

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7
Q

PML epidemiology

A

2-4% of AIDS patients

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8
Q

What drugs confer increased risk for PML?

A

1) natalizumab

2) rituximab

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9
Q

adrenoleukodystrophy complications

A
  • Long-term coma/death

- adrenal gland crisis

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10
Q

adrenoleukodystrophy pathophys

A

buildup of very-long-chain fatty acids in nervous system + adrenal gland + testes

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11
Q

General characteristics of seizures

A

Synchronized, high-frequency neuronal firing

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12
Q

Most common origin of partial seizures

A

medial temporal lobe

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13
Q

partial seizure common scenario

A

preceded by seizure aura.

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14
Q

Simple partial vs. complex partial focal seizures

A

consciousness intact with simple, impaired in complex

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15
Q

status epilepticus cutoff

A

5 minutes

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16
Q

Most common causes of seizures in children…

A

1) genetic
2) infection (febrile)
3) trauma
4) congenital
5) metabolic

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17
Q

Most common causes of seizures in adults…

A

1) tumor
2) trauma
3) stroke
4) infection

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18
Q

Most common causes of seizures in elderly…

A

1) stroke
2) tumor
3) trauma
4) metabolic
5) infection

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19
Q

generalized seizures

A

1) absence
2) myoclonic
3) tonic-clonic
4) tonic
5) atonic

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20
Q

absence seizure characteristics

A

1) 3 Hz
2) no postictal confusion
3) blank stare

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21
Q

tonic clonic (grand mal) presentation

A

alternating stiffening and movement

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22
Q

only headache that’s more common in men?

A

cluster

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23
Q

cluster duration

A

15 min-3 hr; repetitive

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24
Q

cluster associated with?

A

horner syndrome

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25
Q

acute cluster treatment

A

sumatriptan + 100% O2

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26
Q

cluster prophylaxis

A

verapamil

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27
Q

typical tension headache duration

A

Greater than 30 minutes, typically 4-6 hrs. constant.

28
Q

treatment for chronic pain associated with tension headaches?

A

amitriptyline

29
Q

migraine duration

A

4-72 hours

30
Q

migraine pathophys

A

Due to irritation of CN V, meninges, or blood vessels.

31
Q

Molecules related to migraines

A

Release of substance P, calcitonin gene-related peptide, vasoactive peptides.

32
Q

acute migraine treatment

A

1) NSAIDs
2) triptans
3) dihydroergotamine

33
Q

migrane prophylaxis

A

1) lifestyle changes (sleep, exercise, diet)
2) beta blockers
3) calcium channel blockers
4) amitriptyline
5) topiramate
6) valproate

34
Q

trigeminal neuralgia duration

A

less than 1 minute usually

35
Q

more common type of vertigo?

A

peripheral vertigo

36
Q

peripheral vertigo pathophys

A

inner ear etiology (semicircular canal debris, vestibular nerve infection, Meniere disease).

37
Q

How do you test for peripheral vertigo?

A

positional testing leading to delayed horizontal nystagmus.

38
Q

central vertigo pathophys

A

Brain stem or cerebellar lesion (eg stroke affecting vestibular nuclei or posterior fossa tumor).

39
Q

Findings in central vertigo

A

1) directional change of nystagmus
2) skew deviation
3) diplopia
4) dysmetria

40
Q

How do you test for central vertigo?

A

Positional testing –> immediate nystagmus in any direction; may change directions. Focal neurologic findings.

41
Q

Other name for Sturge-Weber syndrome

A

Encephalotrigeminal angiomatosis

42
Q

Sturge-Weber pathophys

A

Congenital, *non-inherited (somatic) developmental anomaly of neural crest derivates due to activating mutation of GNAQ gene.
**sporadic

43
Q

blood vessels affected in storage-weber

A

small (capillary-sized) blood vessels.

44
Q

nevus flammeus

A

non-neoplastic “birthmark” in CN V1/V2 distribution

45
Q

other impt findings in sturge-weber

A

1) ipsilateral leptomeningeal angioma –> leading to seizures/epilepsy
2) early onset glaucoma

46
Q

pathophys of early onset glaucoma in spurge-weber?

A

episcleral hemangioma –> increased IOP –> early-onset glaucoma.

47
Q

What are tram track calcifications?

A

bright spots on imaging on opposing gyri.

48
Q

Increased incidence of what in tuberous sclerosis?

A

1) subepndymal astrocytomas

2) ungual fibromas

49
Q

cardiac complication of tuberous sclerosis?

A

Mitral regurgitation

50
Q

other name for NF1?

A

von Recklinghausen disease

51
Q

origin of neurofibromas

A

neural crest cells

52
Q

mutated tumor suppressor gene in NF1?

A

neurofibromin

53
Q

neurofibromin action

A

negative regulator of RAS

54
Q

hemangioblastoma description

A

high vascularity tumors with hyperchromatic nuclei.

55
Q

where do hemangioblastomas occur in VHL?

A

1) retina
2) *brain stem
3) cerebellum
4) *spine

56
Q

other finding in VHL…

A

angiomatosis

57
Q

angiomatosis?

A

cavernous hemangioma in skin, mucosa, organs

58
Q

GBM tumor type?

A

grade IV astrocytoma

59
Q

GBM staining

A

GFAP (astrocytes)

60
Q

GBM prognosis

A

1-year median survival

61
Q

meningioma origin

A

arachnoid cells

62
Q

meningioma characteristics

A

1) extra-axial (external to brain parenchyma)

2) may have dural attachment (“tail”)

63
Q

meningioma management

A

resection and/or radiosurgery

64
Q

meningioma presentation

A

1) often asymptomatic

2) seizures or focal neurologic signs

65
Q

meningioma pathology

A

1) spindle cells concentrically arranged in a whorled pattern
2) psammoma bodies