Demyelinating Flashcards
MS sx
sensory disturbances are most common initial presentation follow by motor prob and optic neuritis.
MS imaging
juxtacortical lesions. intratentorial lesions- in brainstem and cerebellum. lesions are common in CC.
active lesion shows restricted diffusion
tumefactive MS
present with large area of demyelination. clinical presentation and radiographic appearance mimic a neoplasm or abscess. -may see aphasia, visual field deficits, cog and personality changes, neglect, weakness, sensory deficit. atypical. biopsy needed to dx
optic neuritis (ON)
inflm, demyelinating D of optic N. usually in young adults. blurry vision. decr red. pain with eye mvt. progress over days. get contrast enhanced MRI if enhanced.
bilateral/complete visual loss usual
neuromylitis optica
bilateral inflm of optic N, chiasm, longitudinally extensive myelitis.
ON tx
3d course of IV steroids. follow by oral prednisone taper for 11d.
alexander’s disease
mut in gliala fibrillary acidic protein. in infants with seizures, incr tone, spasticity. rarely present in adult. has relapsing-remitting epic- mimics MS. palatal myoclonus common
NMO criteria
1) optic neuritis
2) myelitis
need 2/3: MRI evidence of contiguous SC lesion >3 set in length, brain MRI at onset non-diagnostic for MS, NMO-IgG seropositivity
NMO patho
NMO ab directed against aquaporin 4 water channel on astrocyte foot processes of BBB. pos in 75% of NMO pt= highly specific but can’t rule out if neg.
ban of brain not uncommon in NMO at sites of high aquaporin 4 expression.
NMO clinical features
like MS has relapses. recover less but no replapse index of relapses. AA> caucasians. high risk for AI.
lesion in posterema are common and present with hiccups and intractable N/V
NMO rads
longitudinally extensive transverse myelitis.
NMO tx
IV steroids and plasmapharesis for severe relapses. some immunosuppresive.
natalizumab induced PML
only ask pt for mir of lesion. ab that prevent lymphocyte from crossing BBB. JC virus.
MS tx
interferon beta, glatiramer acetate, fingolimbod, natalizumb , terifunimode and chemoterapeutic agent mitxontone.
fingolimob
tx MS. oral agent. superior to IM inteferon beta. reversible macular edema dn trasient braycardia with 1st dose.
teriflunimode
tx MS orally by blocking profile and fun of activated T and B lymphocytes. reversibly inhibiting a mito enzyme. no SE
ADEM (adrenoleukodystrophy)
central immune mediated demyelinating inflm disorder occurring during or after a systemic illness.
monophasic . monophasic in 90% pf pt.
ADEM epi. patho
1/3 of pt dx with encephalitis –> MRI pt
from diffuse multifocal inflm. typically pos op pt is nice.
30d btw illnesses. typiclaly ps infectious and report o post vaccination.
empirically tx high dose interred occupationally
ADEM -RF
measles = highest. immunization pt, solid carrots highest risk with ADEM.
improve liver everything. our residents didn’t tell us we did do
ADEM perfusion
HA, lathargy, myalgia, malaise, fever, vomit abrupt.
neuro disturbances respoent encaphalopathy.
ADEM dx
based on MRI.periventricular and CC MS.
ADEM tx
IV steroids. then oral and IVIG steroids.
