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Abstract Details

Search for Anti-Glycine Receptor Antibodies in Stiff-Person Syndrome (SPS)
Movement Disorders
P03 - (-)
057
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: 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: 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: 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, FÂ鶹´«Ã½Ó³»­ (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 Â鶹´«Ã½Ó³»­. 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