Stiff-person syndrome: an autoimmune disease.
Stiff-person syndrome (SPS) is characterized by progressive, usually symmetric rigidity of the axial muscles with superimposed painful spasms precipitated by tactile stimuli, passive stretch, volitional movement of affected or unaffected muscles, startling noises, and emotional stimuli. Electromyography demonstrates continuous normal motor unit potentials in the affected muscles. Both the rigidity and the spasms are relieved by sleep, general anesthesia, myoneural blockade, peripheral nerve blockade, and partially by diazepam. Evidence for an autoimmune etiology of SPS includes its association with other autoimmune diseases and autoantibodies and the presence of antibodies against glutamic acid decarboxylase (GAD) in the cerebrospinal fluid (CSF) of many affected patients. We describe two patients with this syndrome who had GAD antibodies in both CSF and serum. Partial relief of the symptoms in these patients by corticosteroid therapy provides additional evidence of an autoimmune etiology of SPS and of the role of immunotherapy in its treatment.
Blum
P
P
Department of Neurology, Baylor College of Medicine, Houston, Texas 77030.
Jankovic
J
J
D001323:Autoantibodies / Q000032:analysis*; D001327:Autoimmune Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D001418:Baclofen / Q000008:administration & dosage; D003975:Diazepam / Q000008:administration & dosage; D004334:Drug Administration Schedule; D004359:Drug Therapy, Combination; D005260:Female; D005968:Glutamate Decarboxylase / Q000276:immunology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D009127:Muscle Rigidity / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D009129:Muscle Tonus / Q000187:drug effects; D009460:Neurologic Examination; D011241:Prednisone / Q000008:administration & dosage
D001323:Autoantibodies; D005968:Glutamate Decarboxylase; D001418:Baclofen; D003975:Diazepam; D011241:Prednisone