12-04 Diseases of Myelin CPC

Question 1

MS incidence, Male vs. female?

Answer 1

female twice as much

Question 2

geographic distribution

Answer 2

incidence incrs as you get farther from equator

Question 3

most common age at presentation?

Answer 3

generally before 55 y/o

—usually between 20-40 y/o

Question 4

strong association between MS and certain alleles of which protein?

Answer 4

MHC-II (HLA-DRB)

Question 5

Pathophys?

Answer 5

likely autoimmune; assoc w/ viral infx may be b/c:
1. virus weakens BBB—> T-cells enter CNS—> increase diapedesis of other immune cells—> autoimmunity against myelin antigens (e.g. myelin basic protein) is activated—> denuding of axons—> slowed conduction—> sx

Question 6

Common presenting sx of MS

Answer 6

focal weakness, numbness, tingling or unsteadiness on limb; unilateral optic neuritis; diplopia; disequilibrium; or bladder problems

Question 7

relationship of MS to childbirth?

Answer 7

women more likely to flare/present in first 3 mos post partum (though reduce during preg itself)

Question 8

relationship of MS Sx to temperature

Answer 8

heat causes temp worsening of sx

Question 9

What type of MRI scan to image MS?

Answer 9

T2-weighted

Question 10

investigative findings in MS?

Answer 10

1. spinal tap: oligoclonal bands of IgG in 90% pts
2. T1 MRI: HYPOintense “BLACK holes” (perm axonal damage)
3. T1 MRI w/ Gd: inflamm w/ areas of BBB breakdown
4. T2 MRI: lesion load/dz burden (i.e. total # lesions) = HYPERintense WHITE spots

Question 11

clinically isolated syndrome

Answer 11

single clinical event only; incr risk of developing MS
—consider tx as if MS to prevent progression
—consider f/u imagine q6-12 mos for new lesions

Question 12

probable MS

Answer 12

—evidence of multifocal WM dz

—only one attack OR 2+ attack w/ only s/sx of only 1 lesion

Question 13

definite MS

Answer 13

—relapsing-remitting course
—2+ regions of CNS WM affected
—s/sx of 2+ different legions

Question 14

tx of RRMS

Answer 14

—INF-β1a or -β1b” SC/IM 1-3X/wk **flu-like-sx
—glatiramer: (looks like myelin basic prot) SC qD **screen for JC virus b/c incr risk of progressive leukencephalopathy
—fingolimod: PO qD (? inhbit lymphocyte entry into CNS) **bradycardia
—streroids for acute mgmt
—exercise
—bladder mgmt
—sleep! —> amantadine or SSRIs for fatigue

Question 15

MRI w/ Gd

Answer 15

shows lesions less than 30 days old; highlights problems in the BBB