Long-term remission of refractory stiff-man syndrome after treatment with intravenous immunoglobulin.

Abstract

The stiff-man syndrome is a rare neuromuscular disorder characterized by progressive rigidity, stiffness, and intermittent spasm of axial and extremity muscles. Its etiology is unknown. Different therapeutic regimens have been used with variable success. We present a case of refractory stiff-man syndrome, in which the symptoms were successfully controlled by the administration of intravenous immunoglobulin (IVIg). This case gives evidence that IVIg can be a safe and an efficient treatment of refractory stiff-man syndrome. The exact indication for and the cost-effectiveness of IVIg in the treatment of this rare entity remain to be determined.