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Abstract Details

Cerebellar Ataxia as the Initial Presentation in Glutamic Acid Decarboxylase Antibody-positive Stiff Person Syndrome: A Case Series
Autoimmune Neurology
P2 - Poster Session 2 (11:45 AM-12:45 PM)
2-001
A retrospective case series of 3 patients with highly positive antibodies against  Glutamic Acid Decarboxylase (GAD) initially presenting with cerebellar ataxia then subsequently developing stiff person syndrome (SPS)
Elevated GAD antibody levels could present itself in a variety of neurologic conditions including Stiff Person Syndrome (SPS) and cerebellar ataxia. Although intravenous immunoglobulin (IVIG) is the most widely used and preferred treatment for SPS, plasmapheresis (PLEX) and other immunotherapies were used instead.
Retrospective case series
All 3 patients initially presented with dizziness and binocular diplopia. Neuro-ophthalmologic evaluations were remarkable for downbeat nystagmus without findings of body stiffness. Though IVIG was the primary treatment choice for all three, PLEX was done instead because of religious beliefs (Jehovah’s Witness) for the first case and insurance denial for the two other cases. Subsequent to their initial treatment, all three cases were maintained on immune therapy: mycophenolate for the first and second cases, and rituximab for the third case. The second patient had to eventually discontinue mycophenolate due to side effects, and only when she developed SPS was weekly PLEX started. Despite immune therapies, all three patients developed SPS within about 9 years, 3 years, and 4 months, respectively from the time they initially presented with cerebellar ataxia.
Though IVIG is a recognized treatment for SPS associated with GAD antibody, PLEX and other immunotherapies were used instead for GAD antibody-positive cerebellar ataxia. It is unknown whether treatment with IVIG for these patients would have made a difference -- prevented the progression to SPS, or improved their cerebellar ataxia. Though plasmapheresis and immunosuppressants seem to have resulted in some subjective improvement and prevention of worsening of cerebellar ataxia symptoms, it would seem that it is not an effective enough treatment to prevent progression to SPS.
Authors/Disclosures
Ronel Santos, MD (Trinity Health Saint Mary's)
PRESENTER 		An immediate family member of Dr. Santos has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Cochrane. Dr. Santos has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Giarmarco, Mullins & Horton PC. Dr. Santos has or had stock in Gilead Sciences.Dr. Santos has or had stock in Novocure.Dr. Santos has or had stock in Shockwave Medical.Dr. Santos has or had stock in Teva. An immediate family member of Dr. Santos has received research support from Â鶹´«Ã½Ó³»­. The institution of an immediate family member of Dr. Santos has received research support from Trinity Health Saint Mary's Foundation. An immediate family member of Dr. Santos has a non-compensated relationship as a Delegate with Â鶹´«Ã½Ó³»­ Neurology on the Hill that is relevant to Â鶹´«Ã½Ó³»­ interests or activities. An immediate family member of Dr. Santos has a non-compensated relationship as a Participant with Â鶹´«Ã½Ó³»­ Palatucci Advocacy Leadership Forum that is relevant to Â鶹´«Ã½Ó³»­ interests or activities. An immediate family member of Dr. Santos has a non-compensated relationship as a Scholar with Â鶹´«Ã½Ó³»­ Health Equity Symposium Scholarship that is relevant to Â鶹´«Ã½Ó³»­ interests or activities. An immediate family member of Dr. Santos has a non-compensated relationship as a Participant with Â鶹´«Ã½Ó³»­ Emerging Leaders Program that is relevant to Â鶹´«Ã½Ó³»­ interests or activities.
Carlos R. Santos An immediate family member of Mr. Santos has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Cochrane. An immediate family member of Mr. Santos has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Giarmarco, Mullins & Horton PC. The institution of an immediate family member of Mr. Santos has received research support from Saint Mary's Foundation. An immediate family member of Mr. Santos has received research support from Â鶹´«Ã½Ó³»­.
Saad Umar Mr. Umar has nothing to disclose.
Aileen Antonio, MD, FÂ鶹´«Ã½Ó³»­ (Trinity Health Grand Rapids Hauenstein Neurosciences) Dr. Antonio has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Cochrane. An immediate family member of Dr. Antonio has received personal compensation in the range of $500-$4,999 for serving as an Expert Witness for Giamarco, Mullins & Horton PC. The institution of Dr. Antonio has received research support from Saint Mary's Foundation. Dr. Antonio has received research support from Â鶹´«Ã½Ó³»­. Dr. Antonio has received personal compensation in the range of $0-$499 for serving as a Invited Speaker with Michigan State University.
Melanie G. Taylor, MD Dr. Taylor has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Argenx. Dr. Taylor has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Biogen.