Abstract Details

Title Search for Anti-Glycine Receptor Antibodies in Stiff-Person Syndrome (SPS)

Topic Movement Disorders

Presentation(s) P03 - (-)

Poster/Presentation
Number 057

Objective

Background BACKGROUND: SPS is an autoimmune disease causing stiffness in trunk and limp muscles and sudden incapacitating spasms that aggravate stiffness or result in falls. In SPS there is a disturbance in inhibitory GABAergic pathways presumably by autoantibodies against GAD, the main enzyme that synthesizes GABA. As the pathogenetic role of anti-GAD antibodies remains unclear, several other candidate disease-specific autoantigens associated with inhibitory pathways need to be explored. Glycine is a key neurotransmitter in spinal inhibitory interneurons and GlyR antibodies have been described in Encephalomyeliltis with Rigidity, a syndrome with similar phenomenology to SPS.

Design/Methods DESIGN/METHODS: We tested serum samples from the following: 59 patients with SPS and high titer (>20,000 units) of anti-GAD antibodies; 6 patients with other high GAD-antibody associated CNS disorders; 7 patients with low GAD-antibody-associated diseases (both neurological and type-1 diabetes); 14 patients with GAD-negative encephalitis; 20 patients with relapsing-remitting multiple sclerosis (RRMS); and 20 healthy controls. We established a cell-based assay by transfecting HEK293T cells with the glycine receptor cDNA [the GlyR-GFP clone and an anti-GlyR-positive serum used as control, were a kind gift from A. Vincent, Oxford).

Results RESULTS: 8/59 (14%) of SPS patients were positive for anti-GlyR autoimmunity. One SPS patient with a low GAD titer and 1 RRMS patient were also positive. The remaining 65 sera from the other autoimmune neurological diseases or controls were negative.

Conclusions CONCLUSIONS: Anti-GlyR autoimmunity is present in some SPS patients with severe symptoms of stiffness or hyperexcitability. Whether GlyR antibodies identify a distinct subset of SPS patients with excessive spinal excitability or unravel and confirm SPS symptomatology in patients with low GAD titers, is currently under investigation.

Authors/Disclosures
Haralambos Alexopoulos (University of Athens) PRESENTER	No disclosure on file
	No disclosure on file
Marinos C. Dalakas, MD, FAAN (Thomas Jefferson University)	Dr. Dalakas has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Grifols, . Dr. Dalakas has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Dysimmune Diseases Foundation. Dr. Dalakas has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Octapharma. Dr. Dalakas has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for ARGENX. Dr. Dalakas has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for AAN. Dr. Dalakas has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Therapeutic Advances in Neurology (TAND). Dr. Dalakas has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Medlink.
	No disclosure on file

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