Recognizing stiff person syndrome is clinically important. It is uncommon, characterized by body stiffness associated with painful muscle spasms, and varies in location and severity. It is subdivided into stiff trunk versus stiff limb presentation, and as a progressive encephalomyelitis. Stiff person-type syndrome also reflects a paraneoplastic picture. Most patients demonstrate exaggerated lumbar lordosis. Roughly 60% of patients have antiglutamic acid decarboxylase antibodies in the blood and the cerebrospinal fluid. The differential diagnosis includes many severe conditions. There are reports of response to muscle relaxants, immunosuppressants, intravenous gamma globulin, plasma exchange, a number of anticonvulsants, and botulinum toxin.