Inflammatory/Demyelinating Diseases

Question 1

There are two phenotypes of multiple sclerosis. What are they?

Answer 1

Relapsing-remitting (85%): periodic attacks that worsen with time
Progressive (15%): continuous progression from the onset

Question 2

Relapsing-remitting MS can evolve into _____________. What is this called?

Answer 2

progressive; secondary progressive

Question 3

Describe the rough demographics of MS.

Answer 3

More common in women

HIghest incidence in 18 - 45 yo

Question 4

What histologic pattern is (oddly) seen in the meninges of those with MS?

Answer 4

A lymph-node-like structure with B cells

Question 5

Describe the bizarre relationship between duration of MS, age, inflammation, and disability.

Answer 5

In those with MS, inflammation in the CNS usually goes down with age and duration of disease; however, people still tend to get worse (i.e., develop more CNS symptoms), despite lessening of inflammation!

Question 6

Risk of MS is much lower in those who are _______ negative.

Answer 6

EBV

Question 7

What is the monozygotic concordance rate of MS?

Answer 7

30% (dizygotic is only 5%)

Question 8

The _______ genotype has been linked to MS, but the odds ratio is only 1.1.

Answer 8

HLA-DR2

Question 9

What test is very sensitive to MS diagnosis?

Answer 9

MRI –can detect multiple lesions after even one attack; importantly, even one lesion after an MS-like attack is predictive of future attacks.

Question 10

Early MS lesions usually produce _______ symptoms, while later MS lesions usually produce _________ symptoms.

Answer 10

focal; general

Question 11

How do MS lesions appear on T1 and T2 MRIs?

Answer 11

T1: hypointense
T2: hyperbright

Question 12

What percent of those with MS will go on to develop secondary progressive MS?

Answer 12

50%

Question 13

Multiple sclerosis can lead to ___________ transection.

Answer 13

axonal (though axons are mostly spared)

Question 14

What modifiable risk factors have been shown to increase risk of developing MS and of having a worse progression of MS?

Answer 14

Cigarettes
Obesity
EBV
Vitamin D deficiency

Question 15

What brain structure often undergoes marked atrophy in those with MS?

Answer 15

The corpus callosum –a bundle of white matter, which may be attacked in MS

Question 16

What are bad prognostic signs in someone with MS?

Answer 16

Gray matter atrophy
Spinal cord lesions
Cerebellar lesions
High disease burden on MRI

Question 17

Explain the presentation and prevalence of benign and malignant MS.

Answer 17

Benign (10%): rare attacks, no disability

Malignant (5%): rapidly progressive, wheelchair in 5 years

Question 18

How are acute MS attacks treated? What if that treatment fails?

Answer 18

Methylprednisone (solumedrol)

If that fails, perform plasmapharesis.

Question 19

What new therapies are very promising for MS treatment?

Answer 19

Ocrelizumab
Natalizumab (integrin antagonist)
Daclizumab (IL-2 receptor antagonist)

Question 20

Disease-modifying therapies are most effective in __________ patients.

Answer 20

younger

Question 21

How many people have MS in the U.S.?

Answer 21

400,000 (10,000 new cases per year)

Question 22

MS is a disease of the __________ nervous system.

Answer 22

central

Question 23

Primary progressive MS most commonly appears in what age group?

Answer 23

Middle age

Question 24

What destroys myelin in MS?

Answer 24

Lymphocytes, macrophages, and antibody

Question 25

What time/space criteria need to be met to achieve a diagnosis of RRMS and PPMS?

Answer 25

RRMS: two separate lesions occurring for at least 24 hours 30 or more days apart
PPMS: continuous lesions for at least 12 months

Question 26

After a CIS, what percent of people go on to have a second attack?

Answer 26

88%