non-MS demyelinating disease Flashcards
What do NMO and NMOSD stand for?
neuromyelitis optical and neuromyelitis optica spectrum disorders.
what two antibodies are responsible for the pathogenesis of NMO and NMOSD?
anti MOG and AQP4 antibodies, never see with both though-separate diseases.
Where are AQP4 expressed?
On the surface of astrocytes close to the blood-brain barrier (blood vessels)and also in tissues like kidney, stomach and muscles.
Are anti MOG and AQP4 Ab seen in MS patients?
no, not seen in classically presenting MS patients.
What stain is used to show astrocyte degradation (loss) that correlates with AQP4 Ab?
GFAP (brown stain), loss ox astrocytse seen with AQP4 complex formation and complement activation.
What is demyelination like in AQP4 ab NMO and NMOSD?
myelin loss relatively preserved vs MS (secondary to astrocyte damage).
Two other disease that cause loss of astroyctes in the CNS?
ADEM and spinal infarction.
What B cell factors are increased in the CSF of NMO and NMOSD patients with AQP4?
BAFF and APRIl increased, vs MS. Thought to be B cell mediated.
What is general age of onset and ethnicity with higher prevalence (AQP4)?
onset is around 40yo and higher in afro Americans who have a younger presentation too.
Are females or males more likely to get NMO (AQP4)? Does age influence this?
females more likely, but this is greater in adults and highest in eldery vs in children.
what of the symptoms of AQP4 disease are younger and older onset more likely to see?
younger onset is predictive of more severe visual defects.
Older onset is more predictive of transverse myelitis and motor disability.
Older people more likely to have neuropathic pain.
What is common to see in NMO (AQP4)patients alongside this disease?
Many already have other autoimmune diseases, e.g. myasthenia gravis, SLE, hypothyroidism.
What you see on MRIs regarding optics and spinal cord in NMSO (AQP4)?
optic nerve has alot of white signal-inflammation.
long expanded cord lesions extending to medulla indicative of transverse myelitis.
Is reoccurrence likely for NMO patients who are seropositive?
Yes very likely for seropositive patients, not for seronegative NMO patients.
Seropositive also morel likely to have transverse myelitis in future too.
Do you see secondary progression in NMO?
No, secondary progressin not really seen vs MS>
Is neuropathic pain more common in NMOSD (AQP4 patients) vs MS?
Yes, associated with TM..
Atypical NMo patient presentations?
Inflammation of the area postrima, (near medulla), leading to vomiting and uncontrollable hiccups.
Often develops into NMO in future.
Also have atypical brain lesion which are inflamed.
ACute treatment of AQP4 Ab disease?
IV steroids, IVIG and plex.
long term treatment for AGP-4 disease?
steroids, immunosuppressives: mycophenolate and azathioprine.
Rituximab
new agents? (anti Il6R anti C5 and anti CD19).
Are MOG antibodies found in MS?
Used to be thought through poorly sensitive tests, and were used as a mouse model of MS.
What diseases are MOG autoantibodies found in?
ADEM
NMO (ON and TM) (fewer vs AQP4)
unilateral/ bilateral ON
TM/LETM
Are females more susceptible to MOG mediated disease?
No, about 50 50 unlike in AQP4.
Which ethicities more scusceptible to MOG diseases?
caucasians make up the most (unlike AQP4).
Is coexisting autoimmunity seen in MOG disease? Is recovery and lower relapse more common in MOG or AQP4?
co existing autoimmunity less likely in MOG
recovery more likely and fewer relapses vs AQP4.
Where are lesions of TM seen in MOG patients vs AQP4?
in MOG seen lower in cervical and thoracic.
brain lesions and ADEM like disease are much more affected in MOG.
Which has younger age of onset? AQP4 or MOG?
MOG does (~30), AQP4 higher at around 40 and more skewed to the right.
what symptoms of MOG disease relapses more often but has better recovery?
un/bilateral ON and ADEM.
What disabilites can ADEM, LETM and ON lead to?
cognitive impairment,
loss of sphincter control
visual loss.
Acute and long term treatment for MOG disease?
IV prednisolone and IVIG PLEX
Long term, oral steroids for 3-6 months.
