Neuro X Flashcards Preview

STEP > Neuro X > Flashcards

Flashcards in Neuro X Deck (65):
1

deficient enzyme in Krabbe disease

galactocerbosidase

2

what builds up in krabbe's?

1) galactocebroside
2) psychosine

3

Krabbe pathophys

galactocerboside + psychosine destroy myelin sheath.

4

findings in Krabbe's

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

5

Metachromatic leukodystrophy pathophys

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

6

Findings in metachromatic leukodystrophy

1) central and peripheral demyelination with ataxia and dementia

7

PML epidemiology

2-4% of AIDS patients

8

What drugs confer increased risk for PML?

1) natalizumab
2) rituximab

9

adrenoleukodystrophy complications

- Long-term coma/death
- adrenal gland crisis

10

adrenoleukodystrophy pathophys

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

11

General characteristics of seizures

Synchronized, high-frequency neuronal firing

12

Most common origin of partial seizures

medial temporal lobe

13

partial seizure common scenario

preceded by seizure aura.

14

Simple partial vs. complex partial focal seizures

consciousness intact with simple, impaired in complex

15

status epilepticus cutoff

5 minutes

16

Most common causes of seizures in children...

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

17

Most common causes of seizures in adults...

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

18

Most common causes of seizures in elderly...

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

19

generalized seizures

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

20

absence seizure characteristics

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

21

tonic clonic (grand mal) presentation

alternating stiffening and movement

22

only headache that's more common in men?

cluster

23

cluster duration

15 min-3 hr; repetitive

24

cluster associated with?

horner syndrome

25

acute cluster treatment

sumatriptan + 100% O2

26

cluster prophylaxis

verapamil

27

typical tension headache duration

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

28

treatment for chronic pain associated with tension headaches?

amitriptyline

29

migraine duration

4-72 hours

30

migraine pathophys

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

31

Molecules related to migraines

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

32

acute migraine treatment

1) NSAIDs
2) triptans
3) dihydroergotamine

33

migrane prophylaxis

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

34

trigeminal neuralgia duration

less than 1 minute usually

35

more common type of vertigo?

peripheral vertigo

36

peripheral vertigo pathophys

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

37

How do you test for peripheral vertigo?

positional testing leading to delayed horizontal nystagmus.

38

central vertigo pathophys

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

39

Findings in central vertigo

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

40

How do you test for central vertigo?

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

41

Other name for Sturge-Weber syndrome

Encephalotrigeminal angiomatosis

42

Sturge-Weber pathophys

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

43

blood vessels affected in storage-weber

small (capillary-sized) blood vessels.

44

nevus flammeus

non-neoplastic "birthmark" in CN V1/V2 distribution

45

other impt findings in sturge-weber

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

46

pathophys of early onset glaucoma in spurge-weber?

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

47

What are tram track calcifications?

bright spots on imaging on opposing gyri.

48

Increased incidence of what in tuberous sclerosis?

1) subepndymal astrocytomas
2) ungual fibromas

49

cardiac complication of tuberous sclerosis?

Mitral regurgitation

50

other name for NF1?

von Recklinghausen disease

51

origin of neurofibromas

neural crest cells

52

mutated tumor suppressor gene in NF1?

neurofibromin

53

neurofibromin action

negative regulator of RAS

54

hemangioblastoma description

high vascularity tumors with hyperchromatic nuclei.

55

where do hemangioblastomas occur in VHL?

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

56

other finding in VHL...

angiomatosis

57

angiomatosis?

cavernous hemangioma in skin, mucosa, organs

58

GBM tumor type?

grade IV astrocytoma

59

GBM staining

GFAP (astrocytes)

60

GBM prognosis

1-year median survival

61

meningioma origin

arachnoid cells

62

meningioma characteristics

1) extra-axial (external to brain parenchyma)
2) may have dural attachment ("tail")

63

meningioma management

resection and/or radiosurgery

64

meningioma presentation

1) often asymptomatic
2) seizures or focal neurologic signs

65

meningioma pathology

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