Autoantibodies to GABAergic neurons and response to plasmapheresis in stiff‐man syndrome

摘要: Stiff-man syndrome is a rare disorder of unknown etiology in which progressive rigidity, spasms, and continuous motor unit activity may be due to dysfunction GABAergic inhibition alpha neurons. Some patients with this have evidence autoantibodies reactive glutamic acid decarboxylase (GAD). We describe patient stiff-man high titers GAD-like immunoreactivity serum but not spinal fluid. Plasmapheresis resulted lowered antibody titers, decreased exteroceptive reflex responses, reduced activity, marked clinical improvement. Immunohistochemistry using plasma produced specific labeling human experimental animal tissue consistent neurons terminal fields. This response was present samples from more than 200 other patients. These results provide additional an autoimmune mechanism for indicate plasmapheresis beneficial some