[CIS-PAGID] Stiff Person Syndrome

[Nelson, Robert P Jr]

Richard,
Agree with you and Elie re: rituximab.  We've treated two with refractory Stiff Person.  Both adults, one female did not respond.  One male has a remarkable improvement that began about 2-3 months after the 4th weekly dose of 375m/m(2).
Bob

Robert P. Nelson Jr., MD
Professor of Medicine and Pediatrics
Divisions of Hematology/Oncology
535 Barnhill Dr. Ste 473
Indianapolis, IN  46202
Telephone: 317-948-1186
E-mail: ronelson at iupui.edu<mailto:ronelson at iupui.edu>
pager:  317-312-1773

From: pagid-bounces at list.clinimmsoc.org [mailto:pagid-bounces at list.clinimmsoc.org] On Behalf Of Elie Haddad
Sent: Thursday, July 14, 2011 11:56 AM
To: pagid at list.clinimmsoc.org
Cc: Susan Sugerman
Subject: Re: [CIS-PAGID] Stiff Person Syndrome

Richard,
I do agree with your proposition of Rituxan. Also, maybe you should give her subcu-IG instead of IVIG because they induce less systemic reaction and in SPS, systemic reaction (whatever the cause) like any stress may trigger spasms.
Elie


Elie Haddad, MD, PhD;
Professor of Pediatrics, University of Montreal,
Head, Pediatric Immunology and Rheumatology Division,
CHU Sainte-Justine, 3175 Cote Sainte-Catherine
Montreal, QC, H3T 1C5, Canada
Ph: 1 514 345 4713
fax: 1 514 345 4897
e-mail: elie.haddad at umontreal.ca<mailto:elie.haddad at umontreal.ca>




Le 2011-07-13 à 17:34, Richard Wasserman a écrit :


I was asked to see a 12 year old Caucasian female recently diagnosed with Stiff Person Syndrome.

She had longstanding diagnoses of atypical cerebral palsy and CVID.  She functioned fairly will with ongoing neurology, immunology, and physical medicine / rehabilitation specialty care until  May 12, 2010 when she had a severe reaction to her first infusion of Gamunex (excipient glycine) characterized by dramatic worsening of neuromuscular symptoms, she had previously received Octagam. Following this episode, the diagnosis of CP was reassessed and revised to SPS. Since that time, she has developed significant worsening of her neuromuscular complaints and dysautonomia. Autoantibodies, anti-Glutamic Acid Decarboxylase and anti-TPO, have been detected.

She has failed baclofen, immunomodulatory doses of Gamagard, and diazepam. She is currently being plasmapharesed. I have suggested Rituxin.

I don't know the mechanism worsening caused by Gamunex. Any suggestions for additional studies or alternative therapy?

Thank you,
Richard

--
Richard L. Wasserman, MD, PhD
DallasAllergyImmunology
7777 Forest Lane, Suite B-332
Dallas, Texas 75230
Office (972) 566-7788
Fax (972) 566-8837
Cell (214) 697-7211

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