Abstract

Stiff-man syndrome (SMS) is a rare disorder of the central nervous system characterized by painful involuntary stiffening of axial muscles accompanied by spasms. These are often precipitated by tactile or emotional stimuli, volitional movement, and startling noises. Until recently, little has been known about the pathogenesis of SMS. There is evidence now to support the hypothesis that SMS may be an autoimmune disease. This is leading to changes in the way this disease is diagnosed and treated. We describe two patients with SMS and review the pathogenesis, diagnosis, and treatment.