17.6 - Demyelinating disorders

Question 1

meta chromatic leukodystrophy is a due to a deficiency of ______________

Answer 1

arylsulfatase

Question 2

what is the inheritance of metachromatic leukodystrophy?

Answer 2

AR

Question 3

what product accumulates in metachromatic leukodystrophy? where does it accumulate (organelle and cell type)?

Answer 3

• sulfatides

- lysosomes of oligodendrocytes

Question 4

krabbe disease is due to a deficiency of ________________

Answer 4

galactocerebroside

Question 5

what is the inheritance of krabbe disease?

Answer 5

AR

Question 6

what product accumulates in krabbe disease? where does it accumulate (cell type)?

Answer 6

• galactocerebroside

- macrophages

Question 7

adrenoluekodrystrophy is due to impaired addition of ___________ to ___________

Answer 7

• CoA

- long chain fatty acids

Question 8

what is the inheritance of adrenoleukodystrophy?

Answer 8

XR

Question 9

what product accumulates in adrenoluekodystrophy? where does it accumulate?

Answer 9

• fatty acids

- adrenal glands and white matter of brain

Question 10

MS is associated with what HLA?

Answer 10

HLA-DR2

Question 11

treatment of acute MS attacks involves ____________-

Answer 11

high dose steroids

Question 12

treatment of long term MS involves

Answer 12

interferon B

Question 13

subacute sclerosing panencephalitis is a slowly progressing, persistent infection of the brain by what organisms?

Answer 13

measles virus

Question 14

what is the histology of subacute sclerosing panencephalitis?

Answer 14

viral inclusions within neurons (gray matter) and oligodendrocytes (white matter)

Question 15

what is the organism responsible for PML?

Answer 15

JC virus