Demyelinating Diseases - Cohen

Question 1

MC neuro disability young adullts

Answer 1

MS

Question 2

MS

Answer 2

myelin destruction in oligodendrocytes

eventual axon destruction

Question 3

mean age first attack MS

Answer 3

29yo

most often in female 70%

Question 4

epidemiology of MS

Answer 4

away from equator more prevalent

Question 5

HLA of MS

Answer 5

DR15

less D3 and D4

Question 6

path of MS

Answer 6

T cell attack of CNS myelin
-formation of plaques

axons destroyed as disease progressed

antigen on myelin attacked - don’t know it

B cells and macros play role as well

Question 7

gamma interferon

Answer 7

makes MS worse

beta - slow it down

Question 8

beta IFN

Answer 8

make MS better - slow it down

Question 9

majority of MS

Answer 9

relapsing and remitting

relapse - have it again
remit - leave me alone

Question 10

MS clinical course

Answer 10

very variable**

-relapsing/remitting

Question 11

benign MS

Answer 11

rare - small attacks and regain full fxn

Question 12

secondary chronic progressive

Answer 12

just gets worse - after course of relapse/remit

Question 13

kurtzkes rule

Answer 13

90% of disability in first 10 years of initial diagnosis

Question 14

life expectance of MS

Answer 14

shortened only 5-10 years

Question 15

first attack MS

Answer 15

visual loss - double vision
weakness
paresthesia

also incoordination, urination difficulty, depression, dysarthria, tremor

Question 16

gadolinium

Answer 16

shows reent area of demyelination

Question 17

MRI

Answer 17

show lesion in other area than current symptoms

ex / patient has clinical cerebellar defect but MRI shows old lesion in cervical spine

Question 18

diagnosis of MS

Answer 18

when pt has had 2 or more attacks of CNS dysfunction

Question 19

CIS

Answer 19

clinically isolated syndromes

most common first attacks of MS

optic neuritis, vision loss, brainstem/cerebellum signs

spinal cord deficit

evaluated with MRI

Question 20

optic neuritis

Answer 20

50% go on to MS

with loss of vision

demyelinating

affect cranial II

Question 21

pupil afferent defect

Answer 21

shine light in eye - doesn’t react

Question 22

yellow retina

Answer 22

inflammation - pallor

Question 23

optic neuritis pupil reflex

Answer 23

lesion in CN II

shine light in that eye - no constriction either eye

shine light in other eye - constriction of both eyes

Question 24

internuclear ophthalmoplegia

Answer 24

CN III and VI

looking one side - adducting eye cannot reach medial edge of eye

abducting eye goes part way - severe nystagmus

damage to medial longitudinal fasciculus - links CN III and VI

with MS or brain stem stroke

can still converge

Question 25

diagnostic criteria of MS

Answer 25

hx of 2 or more attacks in brain/SC multiple in time and space MRI abnormality in white matter

Question 26

MRI in MS

Answer 26

9 or more hyperintenst T2 lesiosn -one or more gadolinium enhanced lesions 1 lesion of cerebellum at least 3 periventricular lesions also look for cord lesions

Question 27

oligoclonal bands on lumbar puncture

Answer 27

in 90% of MS patients IgG bands high sensitive and specific also increased myelin basic protein

Question 28

tx of MS

Answer 28

work on T cells majority don't undo damage most for relapsing/remitting MS disease modifying treatments

Question 29

beta IFNs

Answer 29

tx of MS limits attacks avenox, betaseron, rebif

Question 30

glatiramer acetate

Answer 30

tx of MS -relapsing/remitting MS reduced further attacks 35% immune system attacks drug instead of myelin - decoy drug

Question 31

natalizumab

Answer 31

monoclonal Ab -prevent T cells to cross BBB tx of MS best tx - reduction in future relapses - 50% can get JC virus - lead to PML - progressive multifocal leukencephalopathy (risk)

Question 32

fingolimod

Answer 32

tx of MS - oral keeps lymphocytes in spleen -less likely to cross BBB

Question 33

corticosteroid tx

Answer 33

can limit severeity and duration of MS relapse

Question 34

neuromyelitis optica

Answer 34

optic neuritis not MS - limited to 2 places -optic nerves and SC -is demyelinating disease NO brain demyelination

Question 35

devic disease

Answer 35

NMO

Question 36

MRI of NMO

Answer 36

damage 3 or more levels

Question 37

respiratory crisis in NMO

Answer 37

if extend to C3 or C4

Question 38

serum Abs to aquaporin channels

Answer 38

NMO more common in optic nerve and SC

Question 39

IgG Ab to aquaporin

Answer 39

in NMO diagnosis

Question 40

tx of NMO

Answer 40

immunosuppression

Question 41

guillan barre syndrome

Answer 41

rapidly worsen paralysis auto immune attack of roots of peripheral and cranial nerves death - resp failure

Question 42

follows infection of resp or GI, surgery, trauma, vaccination

Answer 42

GBS

Question 43

campylobacter jejuni

Answer 43

infection preceding GBS

Question 44

rapid ascending weakness

Answer 44

guillan barre syndrome DTR loss w/in one week

Question 45

GBS

Answer 45

affect sensory and motor nerves

Question 46

diagnosis of GBS

Answer 46

history and PE - rapid motor polyneuropathy - ascending paralysis - sick few weeks before significant elevation of CSF protein cellular chemical dissociation - protein up but WBC normal

Question 47

outcome of GBS

Answer 47

<5% die 90% full recovery - takes weeks to months

Question 48

tx of GBS

Answer 48

plasma exchange - remove protein from blood remove Igs that destroy myelin

Question 49

IV IgG

Answer 49

donor IgG - tx for GBS gets in way of pathologic IgG

Question 50

CIDP

Answer 50

chronic inflammatory demyleninating polyneuropathy chronic GBS slower form - develops 3-6 months tx - oral corticosteroids distinct from GBS, not variant reflexes diminished elevated CSF proteins