022514 1 Myelin Disorders SDL Flashcards Preview

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Flashcards in 022514 1 Myelin Disorders SDL Deck (11):
1

What is multiple sclerosis? Cause?

demyelination due to autoimmune destruction by CD4 and CD8 T cells and macrophages. Occurs randomly throughout time and space, usually in females.

2

Multiple sclerosis lab findings?

increased immunoglobulins in CSF (oligoclonal bands) and plaques

3

What is progressive multifocal leukoencephalopathy (PML)?

demyelination caused by JC virus which infects and kills oligodendrocytes during periods of immunosuppression

4

Progressive multifocal leukoencephalopathy (PML) lab findings?

ring enhancing lesions on MRI
viral inclusions in oligodendrocytes

5

What is acute demyelinating encephalomyelitis (ADEM)? Are symptoms generalized or focal/localizing?

demyelination occuring 1-2 weeks after infection characterized by rapid progression of headache, lethargy, and coma. Symptoms are generalized.

6

What is acute necrotizing hemorrhagic encephalomyelitis?

demyelination presenting similarly to ADEM, but usually occurs after UTI and is much more severe.

7

What is neuromyelitis optica (NMO) (devic disease)? What is diagnostic?

demyelination of optic nerve and spinal cord
autoantibodies against aquaporin receptors

8

What is central pontine myelinolysis? In what situations does it occur?

demyelination of the central pons due to: rapid correction of hyponatremia, alcoholism, or severe electrolyte disturbances

9

What is marchiafava-bignami?

demyelination of corpus callosum

10

metachromatic leukodystrophy
krabbe disease
adrenoleukodystrophy
canavan disease
pelizaeus-Merzbacher disease
vanishing white matter disease
alexander disease

What type of diseases are these?

dysmyelinating

11

What is the difference between demyelination and dysmyelination?

demyelination: normal myelin is lost, typically due to autoimmunity. dysmyelination: myelin is not formed properly or is degraded faster than it is made, typically due to a genetic defect