Demyelinating

Question 1

MS sx

Answer 1

sensory disturbances are most common initial presentation follow by motor prob and optic neuritis.

Question 2

MS imaging

Answer 2

juxtacortical lesions. intratentorial lesions- in brainstem and cerebellum. lesions are common in CC.
active lesion shows restricted diffusion

Question 3

tumefactive MS

Answer 3

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

Question 4

optic neuritis (ON)

Answer 4

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

Question 5

neuromylitis optica

Answer 5

bilateral inflm of optic N, chiasm, longitudinally extensive myelitis.

Question 6

ON tx

Answer 6

3d course of IV steroids. follow by oral prednisone taper for 11d.

Question 7

alexander’s disease

Answer 7

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

Question 8

NMO criteria

Answer 8

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

Question 9

NMO patho

Answer 9

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.

Question 10

NMO clinical features

Answer 10

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

Question 11

NMO rads

Answer 11

longitudinally extensive transverse myelitis.

Question 12

NMO tx

Answer 12

IV steroids and plasmapharesis for severe relapses. some immunosuppresive.

Question 13

natalizumab induced PML

Answer 13

only ask pt for mir of lesion. ab that prevent lymphocyte from crossing BBB. JC virus.

Question 14

MS tx

Answer 14

interferon beta, glatiramer acetate, fingolimbod, natalizumb , terifunimode and chemoterapeutic agent mitxontone.

Question 15

fingolimob

Answer 15

tx MS. oral agent. superior to IM inteferon beta. reversible macular edema dn trasient braycardia with 1st dose.

Question 16

teriflunimode

Answer 16

tx MS orally by blocking profile and fun of activated T and B lymphocytes. reversibly inhibiting a mito enzyme. no SE

Question 17

ADEM (adrenoleukodystrophy)

Answer 17

central immune mediated demyelinating inflm disorder occurring during or after a systemic illness.
monophasic . monophasic in 90% pf pt.

Question 18

ADEM epi. patho

Answer 18

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

Question 19

ADEM -RF

Answer 19

measles = highest. immunization pt, solid carrots highest risk with ADEM.
improve liver everything. our residents didn’t tell us we did do

Question 20

ADEM perfusion

Answer 20

HA, lathargy, myalgia, malaise, fever, vomit abrupt.

neuro disturbances respoent encaphalopathy.

Question 21

ADEM dx

Answer 21

based on MRI.periventricular and CC MS.

Question 22

ADEM tx

Answer 22

IV steroids. then oral and IVIG steroids.