Neuroimmunology

Question 1

tumifactive MS

Answer 1

open ring enhancing without edema
-can have prognosis ~RRMS

Question 2

visual evoked potentials

Answer 2

Measure optic N integrity anterior to optic chiasm

N75-P100 AMPLITUDE are considered the marker of axonal integrity of optic nerve

-P100 LATENCY are considered to be related to demyelination of optic nerve

P100 latency prolonged+normal amplitude=demeylination

P100 low amplitude + normal latency = ischemic optic neuropathy

low amplitude+prolonged latency - optic N compression causing segmental demyelination and axonal loss

Question 3

MS

Answer 3

no muscle atrophy
CSF-can be normal
WBC up to 50, lymph

other w/u: B12, SLE, lyme

axon transection on histology

Question 4

optic neuritis

Answer 4

demyelinating SSEP - latency prolonged, amplitude normal

Question 5

NMO

Answer 5

NMO IgG binds Aq4 water channel in astrocytic foot process at BBB

-progresses with relapses, not btw relapses
-brain usually spared
-natalizumab contraindicated
acute: IV steroids, plasmapharesis
-steroids AZA, cyclophosphamide, ritux maintenance

Question 6

ADEM

Answer 6

monophasic post vaccine/illness
-BG involvement, encephlopathy

Tx: IV methylpred then plasmapharesis

form: hemorrhagic leukoencephalopathy of Weston Hurst -temporal lobes

-can have RRMS, may have been misdiagnosed as ADEM as kid

Question 7

Balo’s concentric sclerosis

Answer 7

progressive demyelinating disease
-concentric rings on MRI like onion skin

Question 8

MS drugs

Answer 8

interferon - category C in pregnancy

glatiramer acetate - can use if pregnancy or lactating

Question 9

Pill MS drugs

Answer 9

All need CBC, LFTs

1. SP1 (sphingosine-1-phosphate R) immune suppressant spingolimod, fingolamod
   No signal to get released-> lymph cells stay in lymph nodes, no lymph on CBC, risk infection
   -EKG once b/c bradycardia, observe x6 hours, VZV infections
   -macular edema, regular eye check up
2. Teraflunamide - inhibits pyramidine synthesis
   -immunomodulator, CBC normal
   -liver failure-regular monitoring,
   hair thinning, category X pregnancy teratogen
3. Fumarates - immunomodulator, unknown MOA
   -dimethyl fumarate - flushing, GI upset, diarrhea, improves after 3 months
   -take with food, check CBC

Question 10

Natalizumab

Answer 10

Ab vs alpha-4 integrin, prevents WBC crossing BBB into CNS
1/1,000 risk PML, higher after 2 yrs and with other immunosuppressants, risk if positive JCV
-risk anaphylaxis ->discontinue if occurs

Contraindicated in NMO

Question 11

PML

Answer 11

PML Sx: aphasia, VF deficits, hemiparesis, cognitive dysfunction
if natalizumab; stop then JCV PCR then plasmapharesis

Question 12

Transverse myelitis

Answer 12

Subacute myelopathy post viral illness, Mycoplasma infection, vaccination, or nothing
-bowel, bladder Sx
-very extensive cord lesion
-vs SLE myelitis - acute and other systemic Sx

Question 13

Dalfampridine

Answer 13

Symptomatic Tx to improve walking in MS
-inhibitor of voltage sensitive K channels

Question 14

R INO

Answer 14

On L gaze: Can’t adduct right eye, with L eye abduction nystagmus
-can be BL in MS

Question 15

Useless hand of Oppenheim

Answer 15

In MS relapse, hand feels useless 2/2 sensory deafferentation with otherwise normal motor fxn

Question 16

Pulfrich’s sign

Answer 16

Visual phenomenon - difficulty following moving objects visually
-lateral motion looks like it has a depth component

Question 17

Alembtuzumab

Answer 17

3rd line after broken through DMARDS
-Ab vs CD52, lysis B and T cells
-risk: hyperthyroidism, ITP (2/2 active B cell return after Tx)

Question 18

MOGAD

Answer 18

maintenance IVIG, if fail satrilizumab
-no sex/race risk factors, equal prevalence
-ON, ADEM, myelitis, cranial neuropathies (trigeminal)
-monophasic or relapsing
-lesions disappear on MRI vs NMO, MS

Dx criteria: core clinical demyelinating event + positive MOG-IgG (clear positive titre>1:100) + supporting info: BL ON, perineural optic shealth, ON edema, >50% ON/MRI features

ON in MOG - steroid responsive, BL, optic disc edema,
they are mostly bilateral and long, although posterior in the former and anterior in the latter.

Question 19

ON in MOG vs NMO vs MS

Answer 19

MS: short, anterior

NMO: long, BL, posterior

MOG: long, BL, anterior with optic disc edema

Question 20

anti-idiotypic antibody

Answer 20

When one antibody binds to an idiotope of another antibody
-Ex: IVIG