[CIS PIDD] [cis-pidd] "incapacitating neurologic disorders temporally associated with IgG products"

[Seppänen Mikko]

Dear Maite,

I switched the topic of the title.The patient below has been treated by our unit, by our neuroimmunologists and by rheumatologists, jointly.

We have a patient with clear (around 4-4.5) IgG hypogamma (IgM, IgA normal, no anti-PnPs measured , were not available at the time). She was prone to invasive infections after surgical interventions.
CVI in development was the thought of physicians caring for her then. IgM and IgA have remained normal.

She developed GADAb+ autoimmune epilepsy (GM+PM) and started to have first signs of autoimmune enecphalitis-like symptoms, and IVIg was started.
She went on to develop axial ataxia, myoclonus, lower limb weakness, lower limb livedo, then full-blown SLE + DLE, also later (when test became available) CSF-NMDAR-IgA-Ab positivity
was noted.

Our hospital district does competitive bidding on IVIg, so she has had several IVIgs attempted.

Though IVIg has been vital to her (she has had almost all biologicals a.s.f. attempted by now as well), she complained of more severe neurologic symptoms during/right after infusion of certain brands and/or lots.
We measured GADAbs from different brands and lots and actually they were present, at times at quite high titres and there was a lot of variation between both brands as well as btw lot to lot. What she told and
what we found had pretty good correlation, but n=1.
GADAb are not supposedly directly pathogenic, might have been a marker of how much other autoimmune Abs as well were in the IVIg???
Patients with GADAbs (like amphiphysin Abs) often have multiple neurologic AI-Abs. I doubt if IVIg started this, but may have provoked something if the patient already has some AI-Ab-production of her own?
Draw CSF and send for wide scan analysis of different intracellular and extracellular/symaptic AI-Abs (for the latter I recommend prof Josip Dalmau).

Just a suggestion, hope it helps?

Sincerely

Mikko Seppänen, MD
Helsinki Finland

________________________________
Lähettäjä: Maite de la Morena [mailto:Maite.delaMorena at UTSouthwestern.edu]
Lähetetty: 7. marraskuuta 2013 2:45
Vastaanottaja: CIS-PIDD
Aihe: RE: [cis-pidd] challenging patient for IVIG/SCIG

I would be curious to ask how many people have seen incapacitating neurologic disorders temporally associated with IgG products

I have recently begun caring for a patient who carries the diagnosis of autoimmune encephalomyelitis-Stiff Person Syndrome with evidence of anti-GAD antibodies.

Mother feels everything started after an unusual reaction to an IVIG product (was changed from Octagam to Gamunex). The reaction occurred at the end of the infusion characterized by tachycardia , then fainting episodes that kept her admitted for two weeks.
Thank you
Maite

Maite de la Morena, MD
Associate Professor of Pediatrics
Division of Allergy and Immunology
University of Texas Southwestern Medical Center in Dallas
5323 Harry HInes Blvd
Dallas, Texas 75390-9063
Phone 214 456-5161
Fax: 214 456-8317
Email: maite.delamorena at utsouthwestern.edu



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