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Stiff-person syndrome (SPS) is characterized by stiffness in trunk and limb muscles, phobic anxiety, and sudden spasms. A disturbance in inhibitory GABAergic pathways, presumably by autoantibodies against GAD (the main GABA-synthesizing enzyme), is considered fundamental for SPS pathogenesis.1 As the precise role of anti-GAD antibodies in SPS remains unclear,2,3 several other candidate disease-specific autoantibodies associated with inhibitory pathways have been explored3,4 or are actively pursued. Recently, McKeon et al.5 described anti-glycine-α1 receptor (GlyR) antibodies in a subset of patients with SPS. Glycine is a neurotransmitter in spinal inhibitory interneurons and GlyR are primarily expressed in the spinal cord, brainstem, and cerebellum. Anti-GlyR antibodies have been associated with progressive encephalomyelitis with rigidity and myoclonus (PERM), a syndrome resembling SPS.6 Our aims were to search for GlyR antibodies in a large number of patients with well-characterized SPS and other CNS autoimmune controls and other GAD-positive disorders; and to correlate anti-GlyR titers with clinical symptomatology using quantitative scales of stiffness and spasms.7
Acknowledgment
The authors thank Dr. Goran Rakocevic and Beverly McElroy, RN, for helping with the care of patients studied at the NIH under Dr. Dalakas's protocols.
Footnotes
Author contributions: Dr. Alexopoulos, Dr. Dalakas: study concept and design. Dr. Alexopoulos, S. Akrivou: Acquisition of data. Dr. Alexopoulos, S. Akrivou, Dr. Dalakas: analysis and interpretation. Dr. Alexopoulos, Dr. Dalakas: drafting and critical revision of the manuscript. Dr. Dalakas: study supervision.
Study funding: No targeted funding reported.
Disclosure: H. Alexopoulos and S. Akrivou report no disclosures. M.C. Dalakas serves on the Scientific Advisory Board or has received speaking honoraria from Baxter, Novartis, Grifols, Servis, and Octapharma. He has acted as a consultant for Therapath. He has received honoraria from TAND (Therapeutic Advances in Neurological Disorders) for serving as Associate Editor. He has received institutional grant support to the University of Athens from Genesis, Merck-Serono, and Novartis. Go to Neurology.org for full disclosures.
- Received May 14, 2013.
- Accepted in final form August 14, 2013.
- © 2013 American Academy of Neurology
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