central nervous system Flashcards

1
Q

holoprosencephaly is due to a defect in-

A

SHH signaling

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2
Q

flow of CSF

A
choroid plexus
lateral ventricles 
interventricular foramen of monro
3rd ventricle
cerebral aquduct
4th ventricle
subarachnoid space (Magendie and Luschka)
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3
Q

most common cause of hydrocephalus in newborns

A

cerebral aqueduct stenosis

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4
Q

dandy-walker malformation

A

failure of cerebellar vermis to form, leads to massive dilation of the 4th ventricle

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5
Q

AC type I

A

low lying cerebellar tonsils- later in life with HA, dizziness

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6
Q

AC type II

A

congenital herniation of tonsils and vermis, presents in infancy with hydrocephalus

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7
Q

AC type I can lead to development of

A

syringomyelia

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8
Q

syringomyelia presentation

A

loss of pain and temp sensation along the arms bilaterally (shaw sign)

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9
Q

expansion of syrinx can lead to 2 complications

A
UE weakness (anterior motor horn)
Horner syndrome (lateral horn)
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10
Q

early sign of ALS

A

weakness and atrophy of hands

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11
Q

ALS is differentiated from syringomyelia in that it lacks

A

sensory sx

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12
Q

familial ALS is due to

A

mutation in SOD-1 that leads to increased free radical damage

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13
Q

degeneration of ___ leads to ataxia in Friederich’s ataxia

A

spinocerebellar tracts

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14
Q

inheritance/gene/problem of Friederich’s ataxia

A

AR
GAA repeat frataxin gene
impaired mitochondrial iron regulation leads to increased free radicals via Fenton reaction

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15
Q

classic triad of Friederich’s ataxia

A

ataxia, cardiomyopathy, kyphoscoliosis

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16
Q

brown-sequard presentation

A

hemisection of the cord

ipsilateral: PT loss at level, UMN signs and loss of vibe/light touch below
contralateral: loss of PT starting 2-3 segments below injury

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17
Q

tabes dorsalis symptoms (3)

A

impaired vibratory sense (destruction of dorsal columns), arygll-robinson pupils (accommodate but don’t react), +rhomberg

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18
Q

fungal meningitis CSF

A

lymphocytes, low glucose

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19
Q

areas of the brain most susceptible to ischemia (4)

A

watershed areas
pyramidal neurons in layers 3/5/6 cortex
pyramidal neurons in hippocampus
Purkinje cells of the cerebellum

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20
Q

thromboembolic stroke- m/c source, type of infarct

A

m/c source- plaque in carotid artery

pale infarct

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21
Q

embolic stroke- m/c source, type of infarct

A

m/c source- atrial fibrillation

hemorrhagic infarct

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22
Q

lacunar stroke- pathophysiology

A

hyaline arteriolosclerosis of the lenticulostriate branches of MCA secondary to chronic hypertension

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23
Q

histologic changes after ischemia- day 0

A

12 hours- red neurons (eosinophilic, pyknosis, cell shrinking)

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24
Q

histologic changes after ischemia- day 1

A

24 hours- liquefactive necrosis
1-3 days- neutrophils
4-7 days- macrophages (microglia)

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25
Q

histologic changes after ischemia- 1 week

A

granulation-like tissue

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26
Q

histologic changes after ischemia- 1 month

A

reactive astrocytes yield gliosis

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27
Q

hemorrhagic strokes are also called

A

intraparenchymal hemorrhages

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28
Q

m/c cause of intraparenchymal hemorrhages

A

rupture of charcot-bouchard aneurysms

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29
Q

charcot-bouchard aneurysm pathophysiology

A

develop secondary to hyaline arteriolosclerosis, complication of long-standing HTN

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30
Q

presentation of intraparenchymal hemorrhage

A

focal neurologic signs, headache

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31
Q

presentation of SAH

A

sudden, severe HA

nuchal rigidity

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32
Q

m/c cause of SAH

A

rupture of berry aneurysm

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33
Q

common sites of berry aneurysm

A

bifurcations- ACA and anterior communicating artery (due to lack of intima media layer)

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34
Q

conditions associated with increased risk of berry aneurysms

A

ADPKD

EDS

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35
Q

long term neurologic deficits related to SAH are secondary to

A

vasospasm (prevent with nimodipine)

36
Q

intraventricular hemorrhage-

A

premature babies (less than 32 weeks) in the first 72 hours of life

37
Q

epidural hematoma

A

temporal bone fracture- rupture of middle meningeal a, blood between dura and skull, lucid interval, lens shaped

38
Q

subdural hematoma

A

rupture of bridging veins, blood between dura and arachnoid, slow bleed, crescent shaped

39
Q

3 populations with increased subdural hematomas

A

shaken baby syndrome
elderly
alcoholic

40
Q

subfalcine herniation + 1 complication

A

cingulate gyrus under falx cerebri, leads to infarction of ACA

41
Q

uncal herniation + 3 complications

A

uncus of temporal lobe under tantrum cerebelli, compression of CN III (fixed dilated pupil, down and out eye), compression of PCA (contralateral homonymous hemianopsia), rupture of paramedian artery (Duret hemorrhage of brainstem)

42
Q

tonsillar herniation + 1 complication

A

cerebellar tonsils into foramen magnum

cardiopulmonary arrest

43
Q

metachromatic leukodystropy (inheritance, deficiency and consequences)

A

AR
arylsulfatase
accumulation of sulfatides in lysosomes

44
Q

Krabbe disease (inheritance, deficiency and consequences)

A

AR
galactocerebrosidase
galactocerebroside accumulates in macrophages

45
Q

Adrenoleukodystrophy

A

X-linked
impaired addition of Co-A to LCFA
accumulation of FA in adrenals and white matter

46
Q

MS HLA association

A

DR2

47
Q

MS lumbar puncture

A

lymphocytosis

Oligoclonal IgG bands

48
Q

central pontine myelinolysis- caused by, results in

A

rapid correction of hyponatremia

locked in syndrome

49
Q

AB amyloid pathophysiology

A

abnormal APP is degraded by b-secretase that leads to b-product = AB amyloid

50
Q

APO increased and decreased risk of AD

A

increased- APO e4

decreased- APO e2

51
Q

familial AD

A

presenilin 1/2

52
Q

neuritic plaques (location, composition)

A

extracellular

AB amyloid and neuritic processes

53
Q

neurofibrillary tangles ((location, composition)

A

intracellular

hyperphosphorylated Tau

54
Q

area of the brain that exhibits greatest atrophy in AD

A

hippocampus

55
Q

4 brain changes in AD:

A

cerebral atrophy
neuritic plaques
neruofibrillary tangles
loss of ACH from nucleus basalis

56
Q

cerebral amyloid angiopathy

A

AB amyloid deposition around blood vessels, increases risk of hemorrhage

57
Q

vascular dementia is characterized by (2)

A

focal neuro deficits

step wise progression

58
Q

Picks disease main impairments (2)

A

language (temporal) and behavior (frontal)

59
Q

lewy bodies contain

A

a-synuclein

60
Q

HD is caused by

A

loss of GABA neurons in caudate nucleus

61
Q

HD inheritance, defect

A

AD

CAG repeat in huntingtin gene on chromosome 4

62
Q

in trinucelotide repeat disorders, anticipation is due to expansion of repeats during-

A

spermatogenesis

63
Q

triad of NPH

A

ataxia
dementia
urinary incontinence

64
Q

pathogenesis of NPH symptoms

A

stretching of descending cortical fibers that line the ventricles

65
Q

pathogenesis of prion diseases

A

prion protein takes an altered B-pleated sheet configuration that cannot be degraded and accumulates

66
Q

CJD is characterized by (4)

A

rapid onset dementia, startle myoclonus, ataxia, sharp waves on EEG

67
Q

characteristic appearance of metastatic brain tumors

A

multiple, well circumscribed lesions at the gray-white junction

68
Q

most common primary CNS tumor of adults

A

glioblastoma multiforme

69
Q

most common primary CNS tumor of children

A

pilocytic astrocytoma

70
Q

whorled pattern with psammoma bodies

A

meningioma

71
Q

perivascular pseudorosettes

A

ependymoma

72
Q

rosenthal fibers

A

pilocytic astrocytoma

73
Q

pesudopallisading tumor cells surrounding areas of necrosis

A

glioblastoma multiforme

74
Q

s100+ with highly cellular areas mixed with mixed regions of low cellularity

A

schwannoma

75
Q

fried-egg appearance

A

oligodendroglioma

76
Q

small, round blue cells in homer-write rosettes

A

medulloblastoma

77
Q

glioblastoma multiforme

A

malignant, astrocytes

butterfly lesion that crosses corpus callosum

78
Q

meningioma

A

ER+ (more common in women)

does not invade cortex, but may compress

79
Q

schwannoma

A

m/c affects CN VIII leading to hearing loss and tinnitus

80
Q

bilateral schwannomas are associated with

A

NF2

81
Q

schwannoma staining

A

S100+ (neural crest cell derived)

82
Q

oligodendroglioma imaging

A

calcified mass, usually in frontal lobe

83
Q

VHL associated masses

A

hemangioblastoma of the cerebellum
renal cysts
RCC

84
Q

pilocytic astrocytoma imaging

A

cystic lesion with mural nodule

85
Q

ependymoma presentation

A

hydrocephalus

86
Q

histologic finding in AIDS dementia

A

microglial nodules