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

[Richard Wasserman]

I don't want to intrude but Susan knows her best. There is a lot going on. 
Richard

Sent from my iPhone
Richard L. Wasserman, MD,PhD
214 697-7211


> On Nov 7, 2013, at 1:12 PM, Maite de la Morena <Maite.delaMorena at UTSouthwestern.edu> wrote:

> 

> Thank you so much for your suggestions. Will communicate with her neurologists

> Maite

> 

> Maite de la Morena, MD

> Associate Professor of Pediatrics

> Division of Allergy and Immunology

> UTSouthwestern Medical Center Dallas

> 5323 Harry Hines Blvd

> Dallas, TX 75390-9063

> Phone: 214 456-5161

> Fax: 214 456-8317

> Email: maite.delamorena at utsouthwestern.edu

> ________________________________________

> From: Seppänen Mikko [Mikko.Seppanen at hus.fi]

> Sent: Thursday, November 07, 2013 12:24 AM

> To: CIS-PIDD

> Subject: [cis-pidd] "incapacitating neurologic disorders temporally associated with IgG products"

> 

> 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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