Inflammatory and Demyelinating Diseases Flashcards
Subtypes of MS
describe relapsing-remitting
sporadic episodes of new or worse symptoms over 2-10 days with varied improvement after 1-6 months
85%
younger age
Subtypes of MS
primary progressive
progressive without true relapses
15%
middle age
Subtypes of MS
secondary progressive
relapsing remitting that converts to progressive
50% of RR MS will convert
Subtypes of MS
relapsing progressing
both relapse and progressive
Subtypes of MS
clinically isolated syndrome
single first attack of apparent demyelination
Subtypes of MS
radiologically isolated syndrome
individuals scanned for non-MS show MS on MRI
most MS what age?
what gender?
what race?
closer or further from equator
MS most common cause of __
15-45 y/o
women
Caucasian
further from equator
CNS inflamm disease
typical clinical symptoms early in MS
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
describe lhermitte’s sign
tinging down spine when flex neck
late symptoms in MS
multifocal
1) early +
2) fatigue
3) sex problems
4) depression
5) cognitive dysfunction
6) pain
7) dysphagia
neuro exam abnormalities
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
describe INO
adduction impairment
MRI changes
where are lesions?
T1 holes means
T1 contrast enhancing lesions means?
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)
MRI changes
T2 bright lesions means?
atrophy occurs where
hyperintense/bright lesions
acute + chronic (volume = burden of disease)
atrophy in decr parenchyma, incr ventricles, incr subarachnoid space
MRI changes
most predictive of disability
T1 holes and atrophy
CSF of MS
normal to slight elev protein 4 oligoclonal bands in CSF not blood
Lipid-specific IgM bands = bad prognostic bands
MS
evoked potentials in primary progressive MS
prolonged potentials in primary progressive MS = demyelination
in brainstem, visual, somatosensory
MS
OCT for?
level fo myelin specific protein
optic nerve damage
elevated myelin protein but nonspecific
MS
lumbar punctures done when
primary progressive MS
drugs for acute attacks
high dose steroids (methylprednisolone +/- prednisone taper)
plasma exchange for severe myelination unresponsive to steroids
Immunotherapy for relapsing remitting MS
ABC-R therapy (avonex, betaseron, copaxone, rebif) IFNbeta 1a IFNbeta 1b glutiramer acetate IFNbeta 1a
mechanism of Fingolimod for relapsing remitting MS
side effects
reacts with sphingosine-1-phos receptors to trap lymphocytes in lymph nodes
bradycardia
macular edema
infection
natalizumab shown to cause
PML by reactivation of JC virus
immunotherapy for secondary progressive MS
IFN beta-1b (betasaron) and
ABC-R
treatment of gait/motor function in MS
dalfampridine (Ampyra)
effect of ABC-R treatment
decr in # of enhancing lesions, (T2 burden)
decr brain atrophy
decr disability progression
most effect use of abc-r tx in sp ms
young pts with short disease duration with inflamm
type IV subtype in white matter only seen where
type IV only seen in primary
progressive MS
active sign of inflamm
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
HLA assoc of MS
HLA-DR2
and IL-7 and IL-2 receptors
environmental causes of MS
1) EBV via B lymphocyte
2) cigarette smoking
3) vitamin D deficiency
4) obesity
5) high salt worse progression but not develop disease
diffeence btwn cns lesions in RMS and PPMS
RRMS = 2 attacks 30+ days apartment
PPMZS = minimum 12 months of progression with disseminated lesions in space
early lesions on T1
hypointesne with leakage of contrast due to BBB damage
enhance for 2-6 weeks then BBB fixed
define dawson’s fingers
lesion perpendicular to long axis of ventricles
over time what happens in brian in T1 MS
holes
atrophy with incr ventricles due to hydrocephalus ex vacuo
spinal cord impact of MS
cigar shaped short lesions (one segments)
contrast enhancing in posterior column
neuromyelitis optica on MRI
3+ or more segments long with swelling and present with pain in mid back
track back up medulla
hypodense –> cord massively swollen
bad prognostic signs in MRI
– Atrophy, especially gray matter – High T1 Burden of Disease (T1 holes) – High T2 Burden of Disease – Posterior fossa lesions – Spinal cord lesions
