neuro Flashcards

1
Q

famililal forms of AD?

A

ApoE2: decreased risk
ApoE4: increased risk
APP, presenilin1, presenilin2: increased risk of early onset

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2
Q

what make up AD senile plaques?

A

extracellular beta-amyloid core

synthesized by cleavage of amyloid precursor protein - APP

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3
Q

what make up AD neurofibrillary tangles?

A

intracellular hyperphosphorylated tau

** # of tangles correlates with degree of dementia

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4
Q

what make up Pick bodies? what dz?

A

frontotemporal dementia

silver-staining spherical tau aggregates

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5
Q

deficiency of which B vitamins can cause dementia?

A

B1
B3
B12

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6
Q

Charcot triad of MS?

A

scanning speech
intention tremor (+ incontinence, INO)
nystagmus

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7
Q

what causes periventricular plaques of MS?

A

oligodendrocyte loss and reactive gliosis

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8
Q

findings in AIDP/Guillan Barre?

A

increased CSF protein with normal cell count

increased protein may cause papilledema

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9
Q

acute disseminated encephalomyelitis?

A

multifocal periventricular inflammation and demyelination after infection or vaccinations

** measles, VZV; rabies/smallpox vaccines

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10
Q

scoliosis and foot deformities (high/flat arches)?

A

Charcot Marie Tooth
AD
defective production of proteins involved in structure/fn of peripheral nn or myelin sheath

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11
Q

peripheral neuropathy, developmental delay, optic atrophy, globoid cells?

A

Krabbe disease
AR lysosomal storage dz - galactocerebrosidase deficiency
galactocerebroside and psychosine destroy myelin sheath

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12
Q

central and peripheral demyelination with ataxia, dementia?

A

metachromatic leukodystrophy
AR lysosomal storage dz - arylsulfatase A deficiency
sulfatide buildup –> impaired production and increased destruction of myelin sheath

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13
Q

CNS demyelination in AIDS patients?

A

PML
due to oligodendrocyte destruction

** increased risk with natalizumab, rituximab

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14
Q

adrenoleukodystrophy?

A

X-linked dz of VLCFA metabolism

–> buildup in nervous system, adrenals, testes

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15
Q

tx of cluster H/A?

A

100% O2

triptans

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16
Q

tx of tension H/A?

A

analgesics
NSAIDs
acetaminophen

chronic: amitryptiline

17
Q

pathogenesis of pain in migraines?

A

CNV/meninges/blood vessel irritation

2/2 release of substance P, calcitonin gene-related peptide, vasoactive peptide

18
Q

port wine stain on face, intellectual disability, glaucoma, epilepsy?

A

Sturge Weber
non-inherited activating mutation of GNAQ gene –> anomaly of neural crest derivatives

ipsilateral leptomeningeal angioma –> sz
episcleral hemangioma –> glaucoma

19
Q

hamartomas, angiofibromas, ash-leaf spots?

A

tuberous sclerosis
AD

    • cardiac findings: MR, rhabdomyoma
    • increased incidence of subependymal astrocytomas, ungual fibromas
20
Q

VHL?

A

hemangioblastomas
angiomatosis
B/L renal cell carcinomas
pheos

21
Q

butterfly glioma, cerebral hemispheres, highly malignant?

A

GBM
astrocytes GFAP +
histo: pseudopalisading pleomorphic cells bordering necrosis/hemorrhage

22
Q

tumor in convexities of hemispheres or parasagittal regions?

A

meningioma
arises from arachnoid cells - extra dural, may have tail
tx: resection/radiosurgery

** histo: spindle cells in whorls, PSAMMOMA BODIES

23
Q

hemangioblastoma?

A

usually cerebellar
if with retinal angioma: think VHL
can produce EPO –> 2ary polycythemia

** histo: close thin-walled capillaries, minimal parenchyma

24
Q

S100+ tumor at cerebellopontine angle?

A

Schwannoma
usually CNVIII
tx: resect or radiosurgery

** B/L vestibular schwannomas = NF2

25
Q

frontal lobe tumor, chicken-wire capillaries, fried-egg cytology?

A

oligodendroglioma
often calcified
rare, slow-growing

26
Q

child with posterior fossa tumor, well-circumscribed?

A
pilocytic astrocytoma
GFAP +
histo: eosinophilic corkscrew fibers = Rosenthal fibers
gross: cystic and solid
benign, good prognosis
27
Q

child with cerebellar tumor, compresses 4th ventricle?

A

medulloblastoma
highly malignant
primitive neuroectodermal tumor
histo: Homer-Wright rosettes, small blue cells

** can send “drop metastases” to spinal cord

28
Q

child with 4th ventricle tumor, +/- hydrocephalus?

A

ependymoma
poor prognosis
histo: perivascular rosettes, rod-shaped belpharoplasts near nucleus

29
Q

bitemporal hemianopsia in adult vs child?

A

adult: pituitary adenoma
child: craniopharyngioma

30
Q

herniation that compresses ACA?

A

subfalcine (cingulate under falx)

31
Q

herniation that causes Duret hemorrhages?

A

transtentorial/downward herniation (central)

rupture of paramedian basilar artery branches

32
Q

herniation that causes blown pupil?

A

uncal herniation
medial temporal lobe –> ipsilateral CN III and PCA
also –> contralateral Kernohan notch compression –> ipsilateral paresis

33
Q

herniation that causes coma/death?

A

cerebellar tonsillar herniation into foramen magnum