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Step 1 Crush 250! - Hans > CNS > Flashcards

CNS Flashcards

1
Q

drugs that inc the risk of JC virus infection?

A

natalizumab (targets alpha 4 integrin), rituximab

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2
Q

mech of PML (progressive multifocal leukoencephalopathy)?

A
  • demyelination of CNS due to destruction of oligodendrocytes
  • associated with JC virus
  • rapidly progressive
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3
Q

another name for forebrain?

A

prosencephalon

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4
Q

another name for midbrain?

A

mesencephalon

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5
Q

another name for hindbrain?

A

rhombencephalon

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6
Q

forebrain (prosencephalon) gives rise to

A
  1. telencephalon

2. diencephalon

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7
Q

midbrain (mesencephalon) gives rise to

A

midbrain

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8
Q

midbrain contains what structure?

A

aqueduct

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9
Q

hindbrain (rhombencephalon) gives rise to

A
  1. metencephalon

2. myelencephalon

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10
Q

metencephalon gives rise to

A
  1. pons

2. cerebellum

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11
Q

myelencephalon gives rise to

A

medulla

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12
Q

notochord induces

A

overlying ectoderm to diff into neuroectoderm and form neural plate

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13
Q

neural plate gives rise to

A
  1. neural tube

2. neural crest cells

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14
Q

notochord becomes

A

nucleus pulposus

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15
Q

alar plate is

A

dorsal and is sensory

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16
Q

basal plate is

A

ventral and is motor

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17
Q

what structures belong to neuroectoderm?

A
  1. CNS neurons,

2. ependymal cells (inner lining of ventricles)

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18
Q

what makes CSF?

A

ependymal cells

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19
Q

2 structures from neural crest?

A
  1. PNS neurons

2. Schwann cells

20
Q

structure from mesoderm?

A

microglia

21
Q

benign childhood tumor associated with “tooth enamel like appearance?”

A

Craniopharyngioma

22
Q

characteristics of craniopharyngioma?

A
  1. may be confused with “pituitary adenoma”
  2. bitemporal hemianopia
  3. most common childhood supratentorial tumor
  4. from Rathke pouch (Adenohypophysis)
  5. calcification common
23
Q

the only supratentorial childhood tumor?

A

craniopharyngioma

24
Q

what path is associated with hyperphosphorylated tau protein?

A

Alzheimer

25
Q

what path is associated with silver staining spherical tau protein?

A

Frontotemporal dementia (Pick’s dz)

26
Q

what path is associated with alpha synuclein defect?

A

Lewy body dementia

27
Q

is ACh inc or dec in Alzheimer?

A

dec

28
Q

what is the cause for communicating hydrocephalus?

A

dec CSF absorption by arachnoid granulations

29
Q

what 2 structures make CSF?

A
  1. ependymal cells

2. choroid plexus

30
Q

what path is associated with arachnoid scarring post-meningitis?

A

communicating hydrocephalus

31
Q

what is normal pressure hydrocephalus?

A
  1. affects elderly
  2. CSF pressue elevated only episodically
  3. no increased subarachnoid space volume
  4. expansion of ventricles –> distorts fibers of corona radiata
32
Q

what is the mech of clinical symptoms in normal pressure hydrocephalus?

A

expansion of ventricles –> distorts fibers of corona radiata leading to urinary incontinence, ataxia, cognitive dysfunction

33
Q

mech of ex vacuo

A

appearance of inc CSF due to dec brain tissue seen in Alzhemier dz, advanced HIV, Pick

34
Q

is ICP inc or dec in communicating hydrocephalus?

A

inc

35
Q

is ICP inc or dec in ex vacuo ventriculomegaly?

A

normal ICP

36
Q

what is Charcot-Marie-Tooth dz?

A

known as hereditary motor and sensory neuropathy, groups of progressive hereditary nerve disorders related to the defective production of proteins involved in the structure and function of peripheral nerves or the myelin sheath

37
Q

what path is associated with scoliosis and foot deformities (high or flat arches)?

A

Charcot-Marie-Tooth dz (HMSN = Hereditary Motor and Sensory Neuropathy)

38
Q

what enzyme is missing in Krabbe?

A

galactocerebrosidase

39
Q

what enzyme is missing in Gaucher dz?

A

glucocerebrosidase

40
Q

what enzyme is missing in metachromatic leukodystrophy?

A

arylsulfatase A def

41
Q

what metabolite is accumulated in metachromatic leukodystrophy?

A

sulfatides

42
Q

what path is associated with central and peripheral demyelination with ataxia, dementia?

A

metachromatic leukodystrophy

43
Q

what path is associated with disrupt metabolism of very long chain fatty acids?

A

adrenoleukodystrophy due to def of peroxisomal acyl CoA synthase

44
Q

what are the symptoms of adrenoleukodystrophy?

A

excessive FA buildup in nervous system, adrenal gland, testes leading to coma/death and adrenal gland crisis

45
Q

what is the sudden wild flailing of 1 arm?

A

hemiballismus (subthalamic nucleus lesion from lacunar stroke due to HTN)

Step 1 Crush 250! - Hans (41 decks)

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