Neuroimmuno

Question 1

MOA of natalizumab

Answer 1

monoclonal Ab against alpha-4-integrin cellular adhesion molecule

binds lymphocytes and stops them from adhering to blood vessels in brain and gut

Question 2

risk of developing PML on natalizumab

Answer 2

1 in 1000

higher with prior immunosuppression, using other immunosuppressants, and after 2 years of continuous use

+ JC virus antibodies even higher risk

Question 3

what to do if anaphylaxis to natalizumab

Answer 3

common - 1 in 50 patients by 4th dose will get

STOP infusion, treat anaphylaxis, DO NOT RESTART natalizumab. It’s over.

Question 4

symptoms of progressive multifocal leukoencephalopathy. MRI? what to do if you suspect PML?

Answer 4

aphasia, visual field deficits, headache, hemiparesis, cognitive dysfunction over weeks

MRI shows focal subcortical lesions in multiple brain areas, may look like MS lesions

if PML suspected, stop natalizumab, check JC virus PCR, and start PLEX to clear remaining natalizumab

Question 5

transverse myelitis - presentations and causes

Answer 5

subacute myelopathy, seems to be immunologic
sensory level, weakness, back pain, bowel/bladder dysfunction

may occur after viral illness, Mycoplasma infection, or vaccination, often not preceded by illness

Question 6

FDA approved to improve walking in patients with MS

Answer 6

dalfampridine

Question 6

FDA approved to improve walking in patients with MS

Answer 6

dalfampridine

Question 7

Oppenheim syndrome in MS

Answer 7

MS patient relapses, can move their hand but think it’s useless.

Question 8

Pulfrich’s sign in MS

Answer 8

can’t follow moving objects visually, lateral motion looks like it has a depth component

Question 9

alemtuzumab MOA and side effects

Answer 9

anti CD-52 - cellular mediated lysis of B and T cells
alemtwozumab
risks of hyperthyroidism and ITP

Question 10

if MS patient on this med wants to become pregnant, need a cholestyramine washout prior to conception because it’s teratogenic

Answer 10

teriflunomide

Question 11

MS drug that is safe in pregnancy

Answer 11

glatimer acetate

Question 12

MS drug that has risk of bradycardia and need 1st dose monitoring

Answer 12

fingolimod

Question 13

Lhermitte’s sign associated with lesion where

Answer 13

cervical cord demyelination

Question 14

middle aged man develops focal seizures involving right face and arm lasting seconds - synchronous jerking of face and arm. remains conscious during these but they dont respond to AEDs. then he becomes confused and has memory loss. diagnosis?

Answer 14

LGI1 limbic encephalitis - faciobrachiodystonic seizures

doesn’t respond to AEDs but responds to immunotherapy

Question 15

which MS med causes flushing and GI upset

Answer 15

dimethyl fumarate

Question 16

which MS med is associated with severe/fatal VZV infection

Answer 16

fingolimod

Question 17

fingolimod MOA

Answer 17

binds sphingosine-1-P receptor on lymphocytes

causes internalization - cells can’t egress from lymph nodes (can’t escape lymph nodes - locked up)

Question 18

dalfampridine side effect

Answer 18

incr risk of seizure esp with renal failure
makes MS patients walk fast tho –

Question 19

MS Med with risk of lipoatrophy

Answer 19

Glatimer acetate

Question 20

Med approved for primary progressive MS

Answer 20

Ocrelizumab anti- CD 20

Question 21

MS Med with risk of neutralizing antibodies

Answer 21

Interferons

Question 22

Multiple demyelinating lesions with hemorrhage

Answer 22

Hurst disease - acute hemorrhagic leukoencephalitis

Question 23

Mitoxantrone

Answer 23

Approved for secondary progressive MS
Can cause dose related heart toxicity
Also alopecia menstrual irregularities

Question 24

Onion bulbs on brain biopsy

Answer 24

Balo’s concentric MS
Alternating bands of demyelination and preserved myelination

Question 25

Krabbe gene mutation

Answer 25

GALC gene —> galactocerebrosidase deficiency

Question 26

Diffuse demyelination with U fiber involvement, accumulation of N acetylasparate

Answer 26

Canavan disease

Question 27

Tigroid demyelination

Answer 27

Tends to refer to Pelizaeus Merzbacher on exam questions
Nystagmus, ataxia, tremor

Question 28

PLP1 gene mutation

Answer 28

Pelizaeus Merzbacher disease
X linked
Tigroid