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Flashcards in CNS Deck (45):
1

drugs that inc the risk of JC virus infection?

natalizumab (targets alpha 4 integrin), rituximab

2

mech of PML (progressive multifocal leukoencephalopathy)?

- demyelination of CNS due to destruction of oligodendrocytes
- associated with JC virus
- rapidly progressive

3

another name for forebrain?

prosencephalon

4

another name for midbrain?

mesencephalon

5

another name for hindbrain?

rhombencephalon

6

forebrain (prosencephalon) gives rise to

1. telencephalon
2. diencephalon

7

midbrain (mesencephalon) gives rise to

midbrain

8

midbrain contains what structure?

aqueduct

9

hindbrain (rhombencephalon) gives rise to

1. metencephalon
2. myelencephalon

10

metencephalon gives rise to

1. pons
2. cerebellum

11

myelencephalon gives rise to

medulla

12

notochord induces

overlying ectoderm to diff into neuroectoderm and form neural plate

13

neural plate gives rise to

1. neural tube
2. neural crest cells

14

notochord becomes

nucleus pulposus

15

alar plate is

dorsal and is sensory

16

basal plate is

ventral and is motor

17

what structures belong to neuroectoderm?

1. CNS neurons,
2. ependymal cells (inner lining of ventricles)

18

what makes CSF?

ependymal cells

19

2 structures from neural crest?

1. PNS neurons
2. Schwann cells

20

structure from mesoderm?

microglia

21

benign childhood tumor associated with "tooth enamel like appearance?"

Craniopharyngioma

22

characteristics of craniopharyngioma?

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

the only supratentorial childhood tumor?

craniopharyngioma

24

what path is associated with hyperphosphorylated tau protein?

Alzheimer

25

what path is associated with silver staining spherical tau protein?

Frontotemporal dementia (Pick's dz)

26

what path is associated with alpha synuclein defect?

Lewy body dementia

27

is ACh inc or dec in Alzheimer?

dec

28

what is the cause for communicating hydrocephalus?

dec CSF absorption by arachnoid granulations

29

what 2 structures make CSF?

1. ependymal cells
2. choroid plexus

30

what path is associated with arachnoid scarring post-meningitis?

communicating hydrocephalus

31

what is normal pressure hydrocephalus?

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

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

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

33

mech of ex vacuo

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

34

is ICP inc or dec in communicating hydrocephalus?

inc

35

is ICP inc or dec in ex vacuo ventriculomegaly?

normal ICP

36

what is Charcot-Marie-Tooth dz?

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

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

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

38

what enzyme is missing in Krabbe?

galactocerebrosidase

39

what enzyme is missing in Gaucher dz?

glucocerebrosidase

40

what enzyme is missing in metachromatic leukodystrophy?

arylsulfatase A def

41

what metabolite is accumulated in metachromatic leukodystrophy?

sulfatides

42

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

metachromatic leukodystrophy

43

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

adrenoleukodystrophy due to def of peroxisomal acyl CoA synthase

44

what are the symptoms of adrenoleukodystrophy?

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

45

what is the sudden wild flailing of 1 arm?

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