Inflammatory and Demyelinating Diseases

Question 1

Subtypes of MS

describe relapsing-remitting

Answer 1

sporadic episodes of new or worse symptoms over 2-10 days with varied improvement after 1-6 months

85%

younger age

Question 2

Subtypes of MS

primary progressive

Answer 2

progressive without true relapses
15%

middle age

Question 3

Subtypes of MS

secondary progressive

Answer 3

relapsing remitting that converts to progressive

50% of RR MS will convert

Question 4

Subtypes of MS

relapsing progressing

Answer 4

both relapse and progressive

Question 5

Subtypes of MS

clinically isolated syndrome

Answer 5

single first attack of apparent demyelination

Question 6

Subtypes of MS

radiologically isolated syndrome

Answer 6

individuals scanned for non-MS show MS on MRI

Question 7

most MS what age?

what gender?

what race?

closer or further from equator

MS most common cause of __

Answer 7

15-45 y/o

women

Caucasian

further from equator

CNS inflamm disease

Question 8

typical clinical symptoms early in MS

Answer 8

unifocal at first (one eye, single cord)

1) paresthesias
2) weakness
3) monocular loss of vision/diplopia
4) gait problems
5) lhermitte’s sign
6) urinary urgency/frequency
7) constipation

Question 9

describe lhermitte’s sign

Answer 9

tinging down spine when flex neck

Question 10

late symptoms in MS

Answer 10

multifocal

1) early +
2) fatigue
3) sex problems
4) depression
5) cognitive dysfunction
6) pain
7) dysphagia

Question 11

neuro exam abnormalities

Answer 11

1) asymm UMN signs
2) decr visual acuity and optic atrophy
4) afferent pupillary defect
5) eye movement problems and INO (adduct impairment)
7) sensory loss
8) cerebellar signs
9) labile affect
10) cog dysfunction

Question 12

describe INO

Answer 12

adduction impairment

Question 13

MRI changes
where are lesions?

T1 holes means

T1 contrast enhancing lesions means?

Answer 13

in periventricular, corpus callosum, juxtacortical regions, spinal cord, brainstem, cerebellum

holes means chronic axonal damage

contrast lesions means breakdown BBB (early sign = 2-6 wks)

Question 14

MRI changes
T2 bright lesions means?

atrophy occurs where

Answer 14

hyperintense/bright lesions

acute + chronic (volume = burden of disease)

atrophy in decr parenchyma, incr ventricles, incr subarachnoid space

Question 15

MRI changes

most predictive of disability

Answer 15

T1 holes and atrophy

Question 16

CSF of MS

Answer 16

normal to slight elev protein 4 oligoclonal bands in CSF not blood

Lipid-specific IgM bands = bad prognostic bands

Question 17

MS

evoked potentials in primary progressive MS

Answer 17

prolonged potentials in primary progressive MS = demyelination
in brainstem, visual, somatosensory

Question 18

MS
OCT for?

level fo myelin specific protein

Answer 18

optic nerve damage

elevated myelin protein but nonspecific

Question 19

MS

lumbar punctures done when

Answer 19

primary progressive MS

Question 20

drugs for acute attacks

Answer 20

high dose steroids (methylprednisolone +/- prednisone taper)

plasma exchange for severe myelination unresponsive to steroids

Question 21

Immunotherapy for relapsing remitting MS

Answer 21

ABC-R therapy (avonex, betaseron, copaxone, rebif)
IFNbeta 1a
IFNbeta 1b
glutiramer acetate
IFNbeta 1a

Question 22

mechanism of Fingolimod for relapsing remitting MS

side effects

Answer 22

reacts with sphingosine-1-phos receptors to trap lymphocytes in lymph nodes

bradycardia
macular edema
infection

Question 23

natalizumab shown to cause

Answer 23

PML by reactivation of JC virus

Question 24

immunotherapy for secondary progressive MS

Answer 24

IFN beta-1b (betasaron) and

ABC-R

Question 25

treatment of gait/motor function in MS

Answer 25

dalfampridine (Ampyra)

Question 26

effect of ABC-R treatment

Answer 26

decr in # of enhancing lesions, (T2 burden)
decr brain atrophy
decr disability progression

Question 27

most effect use of abc-r tx in sp ms

Answer 27

young pts with short disease duration with inflamm

Question 28

type IV subtype in white matter only seen where

Answer 28

type IV only seen in primary

progressive MS

Question 29

active sign of inflamm

Answer 29

myelin degradation products in macrophages

active = dense infiltration of macrophages throughout lesion

Chronic = broad rim of macrophages at edge and macrophages with later stages of myelin degradation products

Question 30

HLA assoc of MS

Answer 30

HLA-DR2

and IL-7 and IL-2 receptors

Question 31

environmental causes of MS

Answer 31

1) EBV via B lymphocyte
2) cigarette smoking
3) vitamin D deficiency
4) obesity
5) high salt worse progression but not develop disease

Question 32

diffeence btwn cns lesions in RMS and PPMS

Answer 32

RRMS = 2 attacks 30+ days apartment

PPMZS = minimum 12 months of progression with disseminated lesions in space

Question 33

early lesions on T1

Answer 33

hypointesne with leakage of contrast due to BBB damage

enhance for 2-6 weeks then BBB fixed

Question 34

define dawson’s fingers

Answer 34

lesion perpendicular to long axis of ventricles

Question 35

over time what happens in brian in T1 MS

Answer 35

holes

atrophy with incr ventricles due to hydrocephalus ex vacuo

Question 36

spinal cord impact of MS

Answer 36

cigar shaped short lesions (one segments)

contrast enhancing in posterior column

Question 37

neuromyelitis optica on MRI

Answer 37

3+ or more segments long with swelling and present with pain in mid back

track back up medulla

hypodense –> cord massively swollen

Question 38

bad prognostic signs in MRI

Answer 38

–  Atrophy, especially gray matter
–  High T1 Burden of Disease (T1 holes) 
–  High T2 Burden of Disease
–  Posterior fossa lesions
–  Spinal cord lesions