neuro

Question 1

famililal forms of AD?

Answer 1

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

Question 2

what make up AD senile plaques?

Answer 2

extracellular beta-amyloid core

synthesized by cleavage of amyloid precursor protein - APP

Question 3

what make up AD neurofibrillary tangles?

Answer 3

intracellular hyperphosphorylated tau

** # of tangles correlates with degree of dementia

Question 4

what make up Pick bodies? what dz?

Answer 4

frontotemporal dementia

silver-staining spherical tau aggregates

Question 5

deficiency of which B vitamins can cause dementia?

Answer 5

B1
B3
B12

Question 6

Charcot triad of MS?

Answer 6

scanning speech
intention tremor (+ incontinence, INO)
nystagmus

Question 7

what causes periventricular plaques of MS?

Answer 7

oligodendrocyte loss and reactive gliosis

Question 8

findings in AIDP/Guillan Barre?

Answer 8

increased CSF protein with normal cell count

increased protein may cause papilledema

Question 9

acute disseminated encephalomyelitis?

Answer 9

multifocal periventricular inflammation and demyelination after infection or vaccinations

** measles, VZV; rabies/smallpox vaccines

Question 10

scoliosis and foot deformities (high/flat arches)?

Answer 10

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

Question 11

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

Answer 11

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

Question 12

central and peripheral demyelination with ataxia, dementia?

Answer 12

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

Question 13

CNS demyelination in AIDS patients?

Answer 13

PML
due to oligodendrocyte destruction

** increased risk with natalizumab, rituximab

Question 14

adrenoleukodystrophy?

Answer 14

X-linked dz of VLCFA metabolism

–> buildup in nervous system, adrenals, testes

Question 15

tx of cluster H/A?

Answer 15

100% O2

triptans

Question 16

tx of tension H/A?

Answer 16

analgesics
NSAIDs
acetaminophen

chronic: amitryptiline

Question 17

pathogenesis of pain in migraines?

Answer 17

CNV/meninges/blood vessel irritation

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

Question 18

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

Answer 18

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

ipsilateral leptomeningeal angioma –> sz
episcleral hemangioma –> glaucoma

Question 19

hamartomas, angiofibromas, ash-leaf spots?

Answer 19

tuberous sclerosis
AD

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

Question 20

VHL?

Answer 20

hemangioblastomas
angiomatosis
B/L renal cell carcinomas
pheos

Question 21

butterfly glioma, cerebral hemispheres, highly malignant?

Answer 21

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

Question 22

tumor in convexities of hemispheres or parasagittal regions?

Answer 22

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

** histo: spindle cells in whorls, PSAMMOMA BODIES

Question 23

hemangioblastoma?

Answer 23

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

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

Question 24

S100+ tumor at cerebellopontine angle?

Answer 24

Schwannoma
usually CNVIII
tx: resect or radiosurgery

** B/L vestibular schwannomas = NF2

Question 25

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

Answer 25

oligodendroglioma often calcified rare, slow-growing

Question 26

child with posterior fossa tumor, well-circumscribed?

Answer 26

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

Question 27

child with cerebellar tumor, compresses 4th ventricle?

Answer 27

medulloblastoma highly malignant primitive neuroectodermal tumor histo: Homer-Wright rosettes, small blue cells ** can send "drop metastases" to spinal cord

Question 28

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

Answer 28

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

Question 29

bitemporal hemianopsia in adult vs child?

Answer 29

adult: pituitary adenoma child: craniopharyngioma

Question 30

herniation that compresses ACA?

Answer 30

subfalcine (cingulate under falx)

Question 31

herniation that causes Duret hemorrhages?

Answer 31

transtentorial/downward herniation (central) | rupture of paramedian basilar artery branches

Question 32

herniation that causes blown pupil?

Answer 32

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

Question 33

herniation that causes coma/death?

Answer 33

cerebellar tonsillar herniation into foramen magnum