Demyelinating Diseases - Cohen Flashcards
MC neuro disability young adullts
MS
MS
myelin destruction in oligodendrocytes
eventual axon destruction
mean age first attack MS
29yo
most often in female 70%
epidemiology of MS
away from equator more prevalent
HLA of MS
DR15
less D3 and D4
path of MS
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
gamma interferon
makes MS worse
beta - slow it down
beta IFN
make MS better - slow it down
majority of MS
relapsing and remitting
relapse - have it again
remit - leave me alone
MS clinical course
very variable**
-relapsing/remitting
benign MS
rare - small attacks and regain full fxn
secondary chronic progressive
just gets worse - after course of relapse/remit
kurtzkes rule
90% of disability in first 10 years of initial diagnosis
life expectance of MS
shortened only 5-10 years
first attack MS
visual loss - double vision
weakness
paresthesia
also incoordination, urination difficulty, depression, dysarthria, tremor
gadolinium
shows reent area of demyelination
MRI
show lesion in other area than current symptoms
ex / patient has clinical cerebellar defect but MRI shows old lesion in cervical spine
diagnosis of MS
when pt has had 2 or more attacks of CNS dysfunction
CIS
clinically isolated syndromes
most common first attacks of MS
optic neuritis, vision loss, brainstem/cerebellum signs
spinal cord deficit
evaluated with MRI
optic neuritis
50% go on to MS
with loss of vision
demyelinating
affect cranial II
pupil afferent defect
shine light in eye - doesn’t react
yellow retina
inflammation - pallor
optic neuritis pupil reflex
lesion in CN II
shine light in that eye - no constriction either eye
shine light in other eye - constriction of both eyes
internuclear ophthalmoplegia
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
diagnostic criteria of MS
hx of 2 or more attacks in brain/SC
multiple in time and space
MRI abnormality in white matter
MRI in MS
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
oligoclonal bands on lumbar puncture
in 90% of MS patients
IgG bands
high sensitive and specific
also increased myelin basic protein
tx of MS
work on T cells majority
don’t undo damage
most for relapsing/remitting MS
disease modifying treatments
beta IFNs
tx of MS
limits attacks
avenox, betaseron, rebif
glatiramer acetate
tx of MS
-relapsing/remitting MS
reduced further attacks 35%
immune system attacks drug instead of myelin - decoy drug
natalizumab
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)
fingolimod
tx of MS - oral
keeps lymphocytes in spleen
-less likely to cross BBB
corticosteroid tx
can limit severeity and duration of MS relapse
neuromyelitis optica
optic neuritis
not MS - limited to 2 places
-optic nerves and SC
-is demyelinating disease
NO brain demyelination
devic disease
NMO
MRI of NMO
damage 3 or more levels
respiratory crisis in NMO
if extend to C3 or C4
serum Abs to aquaporin channels
NMO
more common in optic nerve and SC
IgG Ab to aquaporin
in NMO diagnosis
tx of NMO
immunosuppression
guillan barre syndrome
rapidly worsen paralysis
auto immune attack of roots of peripheral and cranial nerves
death - resp failure
follows infection of resp or GI, surgery, trauma, vaccination
GBS
campylobacter jejuni
infection preceding GBS
rapid ascending weakness
guillan barre syndrome
DTR loss w/in one week
GBS
affect sensory and motor nerves
diagnosis of GBS
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
outcome of GBS
<5% die
90% full recovery - takes weeks to months
tx of GBS
plasma exchange - remove protein from blood
remove Igs that destroy myelin
IV IgG
donor IgG - tx for GBS
gets in way of pathologic IgG
CIDP
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
