Neuroimmuno Flashcards
MOA of natalizumab
monoclonal Ab against alpha-4-integrin cellular adhesion molecule
binds lymphocytes and stops them from adhering to blood vessels in brain and gut
risk of developing PML on natalizumab
1 in 1000
higher with prior immunosuppression, using other immunosuppressants, and after 2 years of continuous use
+ JC virus antibodies even higher risk
what to do if anaphylaxis to natalizumab
common - 1 in 50 patients by 4th dose will get
STOP infusion, treat anaphylaxis, DO NOT RESTART natalizumab. It’s over.
symptoms of progressive multifocal leukoencephalopathy. MRI? what to do if you suspect PML?
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
transverse myelitis - presentations and causes
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
FDA approved to improve walking in patients with MS
dalfampridine
FDA approved to improve walking in patients with MS
dalfampridine
Oppenheim syndrome in MS
MS patient relapses, can move their hand but think it’s useless.
Pulfrich’s sign in MS
can’t follow moving objects visually, lateral motion looks like it has a depth component
alemtuzumab MOA and side effects
anti CD-52 - cellular mediated lysis of B and T cells
alemtwozumab
risks of hyperthyroidism and ITP
if MS patient on this med wants to become pregnant, need a cholestyramine washout prior to conception because it’s teratogenic
teriflunomide
MS drug that is safe in pregnancy
glatimer acetate
MS drug that has risk of bradycardia and need 1st dose monitoring
fingolimod
Lhermitte’s sign associated with lesion where
cervical cord demyelination
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?
LGI1 limbic encephalitis - faciobrachiodystonic seizures
doesn’t respond to AEDs but responds to immunotherapy
which MS med causes flushing and GI upset
dimethyl fumarate
which MS med is associated with severe/fatal VZV infection
fingolimod
fingolimod MOA
binds sphingosine-1-P receptor on lymphocytes
causes internalization - cells can’t egress from lymph nodes (can’t escape lymph nodes - locked up)
dalfampridine side effect
incr risk of seizure esp with renal failure
makes MS patients walk fast tho –
MS Med with risk of lipoatrophy
Glatimer acetate
Med approved for primary progressive MS
Ocrelizumab anti- CD 20
MS Med with risk of neutralizing antibodies
Interferons
Multiple demyelinating lesions with hemorrhage
Hurst disease - acute hemorrhagic leukoencephalitis
Mitoxantrone
Approved for secondary progressive MS
Can cause dose related heart toxicity
Also alopecia menstrual irregularities
Onion bulbs on brain biopsy
Balo’s concentric MS
Alternating bands of demyelination and preserved myelination
Krabbe gene mutation
GALC gene —> galactocerebrosidase deficiency
Diffuse demyelination with U fiber involvement, accumulation of N acetylasparate
Canavan disease
Tigroid demyelination
Tends to refer to Pelizaeus Merzbacher on exam questions
Nystagmus, ataxia, tremor
PLP1 gene mutation
Pelizaeus Merzbacher disease
X linked
Tigroid
