10.10 Demyelinating Diseases

Question 1

multiple sclerosis has linkage to

Answer 1

HLA-DR2

Question 2

oligoclonal bands in CSF

Answer 2

multiple sclerosis

Question 3

myelin loss w/ finger like protrusions

Answer 3

multiple sclerosis

Question 4

cells you see in all demyelinating diseases

Answer 4

Creutzfeldt cells

Question 5

see shadow plaques and inactive plaques

Answer 5

multiple sclerosis

Question 6

see all active plaques

Answer 6

acute / Marburg variant of multiple sclerosis

Question 7

only see optic neuritis and spinal cord demyelination

Answer 7

neuromyelitis optica / devic disease

Question 8

antibodies to aquaporins

Answer 8

neuromyelitis optica / devic disease

Question 9

child after viral illness or immunization gets myelin loss around blood vessels

Answer 9

acute disseminated encephalomyelitis

Question 10

occurs after viral illness, immunization, or exposure to drugs, get myelin loss around blood vessels, vascular necrosis, edema, petechial hemorrhage in white matter, neutrophils, and is deadly

Answer 10

acute hemorrhagic leukoencephalitis / Weston-Hurst Disease

Question 11

inherited defect in myelination resulting in hypo or dys myelination

Answer 11

leukodystrophies

Question 12

enzyme deficiency of metachromatic leukodystrophy

Answer 12

arylsulfatase A

Question 13

enzyme deficiency of Krabbe disease

Answer 13

galactocerebroside - beta galactosidase

Question 14

enzyme deficiency of adrenoleukodystrophy

Answer 14

peroxisome enzymes

Question 15

enzyme deficiency of Alexander disease

Answer 15

GFAP gene

Question 16

enzyme deficiency of Pelizaeus-Merzbacher disease

Answer 16

proteolipid protein

Question 17

enzyme deficiency of Canavan disease

Answer 17

aspartoacylase

Question 18

enzyme deficiency of vanishing white matter disease

Answer 18

translation initiation factor eIF2B

Question 19

arylsulfatase A deficiency

Answer 19

metachromatic leukodystrophy

Question 20

galactocerebroside - beta galactosidase

Answer 20

Krabbe disease

Question 21

GFAP gene deficiency

Answer 21

Alexander disease

Question 22

proteolipid protein deficiency

Answer 22

Pelizaeus-Merzbacher disease

Question 23

aspartoacylase deficiency

Answer 23

Canavan disease

Question 24

translation initiation factor eIF2B deficiency

Answer 24

Vanishing white matter disease

Question 25

prismatic or tuffstone inclusions

Answer 25

metachromatic leukodystrophy

Question 26

demyelination and multinucleated globoid cells

Answer 26

krabbe disease

Question 27

accumulate very long chain fatty acids

Answer 27

adrenoleukodystrophy

Question 28

adrenal dysfunction and myelin loss especially in posterior brain

Answer 28

adrenoleukodystrophy

Question 29

cleft-like lamellar inclusions

Answer 29

adrenoleukodystrophy

Question 30

looks like MS but in young boy with adrenal problems

Answer 30

adrenoleukodystrophy

Question 31

demyelination and many Rosenthal fibers

Answer 31

Alexander disease

Question 32

tigroid demyelination

Answer 32

Pelizaeus-Merzbacher disease

Question 33

Alzheimer type 2 cells

Answer 33

Canavan disease

Question 34

punched out holes of white matter

Answer 34

Canavan disease

Question 35

white matter loss with foamy oligodenderocytes

Answer 35

vanishing white matter disease

Question 36

oligodendrocyte with intranuclear inclusion

Answer 36

PML