Disorders of Myelination

Question 1

what is lhermitte sign?

what does it indicate?

Answer 1

electrical sensation radiating down the spine when the neck is passively flexed

-spinal cord disease

Question 2

Approximately how many people in the US have MS?

Answer 2

250k

Question 3

what can also be prescribed prophylacticly with glucocorticoid use?

Answer 3

Ranitidine - steroids can cause GI upset

Question 4

Canavan disease results from defect in N-acetylaspartic acid metabolism, how does this affect the brain?

(macrocephaly vs anencephaly etc)

Answer 4

macrocephaly

increase in brain volume and weight

Question 5

What commonly occurs with the bladder in MS patients?

Answer 5

spastic (upper motor neuron) bladder

contractility is good but they cant hold it (distensibility is bad) results in urgency/incontinence

Question 6

What symptoms will you see with neuromyelitis optica?

Answer 6

symptoms of bilateral optic neuritis in association with a transverse myelitis.

Question 7

familial patterns of development of blind spot with defects in mitochondrial DNA makes you think of what disease?

Answer 7

Hereditary Leber optic atrophy

Question 8

What will be seen on pathology of Pelizaeus-Merzbacher disease

Answer 8

Sudan-staining characteristics of involved white matter

this is a demyelinating disease affecting children usually lethal in first few years