non-MS demyelinating disease

Question 1

What do NMO and NMOSD stand for?

Answer 1

neuromyelitis optical and neuromyelitis optica spectrum disorders.

Question 2

what two antibodies are responsible for the pathogenesis of NMO and NMOSD?

Answer 2

anti MOG and AQP4 antibodies, never see with both though-separate diseases.

Question 3

Where are AQP4 expressed?

Answer 3

On the surface of astrocytes close to the blood-brain barrier (blood vessels)and also in tissues like kidney, stomach and muscles.

Question 4

Are anti MOG and AQP4 Ab seen in MS patients?

Answer 4

no, not seen in classically presenting MS patients.

Question 5

What stain is used to show astrocyte degradation (loss) that correlates with AQP4 Ab?

Answer 5

GFAP (brown stain), loss ox astrocytse seen with AQP4 complex formation and complement activation.

Question 6

What is demyelination like in AQP4 ab NMO and NMOSD?

Answer 6

myelin loss relatively preserved vs MS (secondary to astrocyte damage).

Question 7

Two other disease that cause loss of astroyctes in the CNS?

Answer 7

ADEM and spinal infarction.

Question 8

What B cell factors are increased in the CSF of NMO and NMOSD patients with AQP4?

Answer 8

BAFF and APRIl increased, vs MS. Thought to be B cell mediated.

Question 9

What is general age of onset and ethnicity with higher prevalence (AQP4)?

Answer 9

onset is around 40yo and higher in afro Americans who have a younger presentation too.

Question 10

Are females or males more likely to get NMO (AQP4)? Does age influence this?

Answer 10

females more likely, but this is greater in adults and highest in eldery vs in children.

Question 11

what of the symptoms of AQP4 disease are younger and older onset more likely to see?

Answer 11

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.

Question 12

What is common to see in NMO (AQP4)patients alongside this disease?

Answer 12

Many already have other autoimmune diseases, e.g. myasthenia gravis, SLE, hypothyroidism.

Question 13

What you see on MRIs regarding optics and spinal cord in NMSO (AQP4)?

Answer 13

optic nerve has alot of white signal-inflammation.

long expanded cord lesions extending to medulla indicative of transverse myelitis.

Question 14

Is reoccurrence likely for NMO patients who are seropositive?

Answer 14

Yes very likely for seropositive patients, not for seronegative NMO patients.

Seropositive also morel likely to have transverse myelitis in future too.

Question 15

Do you see secondary progression in NMO?

Answer 15

No, secondary progressin not really seen vs MS>

Question 16

Is neuropathic pain more common in NMOSD (AQP4 patients) vs MS?

Answer 16

Yes, associated with TM..

Question 17

Atypical NMo patient presentations?

Answer 17

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.

Question 18

ACute treatment of AQP4 Ab disease?

Answer 18

IV steroids, IVIG and plex.

Question 19

long term treatment for AGP-4 disease?

Answer 19

steroids, immunosuppressives: mycophenolate and azathioprine.
Rituximab
new agents? (anti Il6R anti C5 and anti CD19).

Question 20

Are MOG antibodies found in MS?

Answer 20

Used to be thought through poorly sensitive tests, and were used as a mouse model of MS.

Question 21

What diseases are MOG autoantibodies found in?

Answer 21

ADEM
NMO (ON and TM) (fewer vs AQP4)
unilateral/ bilateral ON
TM/LETM

Question 22

Are females more susceptible to MOG mediated disease?

Answer 22

No, about 50 50 unlike in AQP4.

Question 23

Which ethicities more scusceptible to MOG diseases?

Answer 23

caucasians make up the most (unlike AQP4).

Question 24

Is coexisting autoimmunity seen in MOG disease? Is recovery and lower relapse more common in MOG or AQP4?

Answer 24

co existing autoimmunity less likely in MOG

recovery more likely and fewer relapses vs AQP4.

Question 25

Where are lesions of TM seen in MOG patients vs AQP4?

Answer 25

in MOG seen lower in cervical and thoracic.

brain lesions and ADEM like disease are much more affected in MOG.

Question 26

Which has younger age of onset? AQP4 or MOG?

Answer 26

MOG does (~30), AQP4 higher at around 40 and more skewed to the right.

Question 27

what symptoms of MOG disease relapses more often but has better recovery?

Answer 27

un/bilateral ON and ADEM.

Question 28

What disabilites can ADEM, LETM and ON lead to?

Answer 28

cognitive impairment,
loss of sphincter control
visual loss.

Question 29

Acute and long term treatment for MOG disease?

Answer 29

IV prednisolone and IVIG PLEX

Long term, oral steroids for 3-6 months.