CNS

Question 1

drugs that inc the risk of JC virus infection?

Answer 1

natalizumab (targets alpha 4 integrin), rituximab

Question 2

mech of PML (progressive multifocal leukoencephalopathy)?

Answer 2

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

Question 3

another name for forebrain?

Answer 3

prosencephalon

Question 4

another name for midbrain?

Answer 4

mesencephalon

Question 5

another name for hindbrain?

Answer 5

rhombencephalon

Question 6

forebrain (prosencephalon) gives rise to

Answer 6

1. telencephalon

2. diencephalon

Question 7

midbrain (mesencephalon) gives rise to

Answer 7

midbrain

Question 8

midbrain contains what structure?

Answer 8

aqueduct

Question 9

hindbrain (rhombencephalon) gives rise to

Answer 9

1. metencephalon

2. myelencephalon

Question 10

metencephalon gives rise to

Answer 10

1. pons

2. cerebellum

Question 11

myelencephalon gives rise to

Answer 11

medulla

Question 12

notochord induces

Answer 12

overlying ectoderm to diff into neuroectoderm and form neural plate

Question 13

neural plate gives rise to

Answer 13

1. neural tube

2. neural crest cells

Question 14

notochord becomes

Answer 14

nucleus pulposus

Question 15

alar plate is

Answer 15

dorsal and is sensory

Question 16

basal plate is

Answer 16

ventral and is motor

Question 17

what structures belong to neuroectoderm?

Answer 17

1. CNS neurons,

2. ependymal cells (inner lining of ventricles)

Question 18

what makes CSF?

Answer 18

ependymal cells

Question 19

2 structures from neural crest?

Answer 19

1. PNS neurons

2. Schwann cells

Question 20

structure from mesoderm?

Answer 20

microglia

Question 21

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

Answer 21

Craniopharyngioma

Question 22

characteristics of craniopharyngioma?

Answer 22

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

Question 23

the only supratentorial childhood tumor?

Answer 23

craniopharyngioma

Question 24

what path is associated with hyperphosphorylated tau protein?

Answer 24

Alzheimer

Question 25

what path is associated with silver staining spherical tau protein?

Answer 25

Frontotemporal dementia (Pick's dz)

Question 26

what path is associated with alpha synuclein defect?

Answer 26

Lewy body dementia

Question 27

is ACh inc or dec in Alzheimer?

Answer 27

dec

Question 28

what is the cause for communicating hydrocephalus?

Answer 28

dec CSF absorption by arachnoid granulations

Question 29

what 2 structures make CSF?

Answer 29

1. ependymal cells | 2. choroid plexus

Question 30

what path is associated with arachnoid scarring post-meningitis?

Answer 30

communicating hydrocephalus

Question 31

what is normal pressure hydrocephalus?

Answer 31

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

Question 32

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

Answer 32

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

Question 33

mech of ex vacuo

Answer 33

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

Question 34

is ICP inc or dec in communicating hydrocephalus?

Answer 34

inc

Question 35

is ICP inc or dec in ex vacuo ventriculomegaly?

Answer 35

normal ICP

Question 36

what is Charcot-Marie-Tooth dz?

Answer 36

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

Question 37

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

Answer 37

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

Question 38

what enzyme is missing in Krabbe?

Answer 38

galactocerebrosidase

Question 39

what enzyme is missing in Gaucher dz?

Answer 39

glucocerebrosidase

Question 40

what enzyme is missing in metachromatic leukodystrophy?

Answer 40

arylsulfatase A def

Question 41

what metabolite is accumulated in metachromatic leukodystrophy?

Answer 41

sulfatides

Question 42

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

Answer 42

metachromatic leukodystrophy

Question 43

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

Answer 43

adrenoleukodystrophy due to def of peroxisomal acyl CoA synthase

Question 44

what are the symptoms of adrenoleukodystrophy?

Answer 44

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

Question 45

what is the sudden wild flailing of 1 arm?

Answer 45

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