Treatment of Stiff Man Syndrome with IVIG

Source: Barker RA, Marsden CD. Successful treatment of stiff man syndrome with intravenous immunoglobulin. J Neurol Neurosurg Neuropsychiat 1997;62:426.

In a short letter, barker and marsden remind us that parenteral immunoglobulin (IG) can ameliorate classic autoantibody stiff man syndrome. They treated a 43-year-old man who had classic stiff man symptoms and EMG findings involving the lower trunk and legs. He had insulin dependent diabetes, his mother had thyrotoxicosis, his maternal grandmother had diabetes mellitus, and an uncle had pernicious anemia. The patient had a weakly positive antiparietal cell (stomach) antibody and serum anti-GAD titer of 1:500. Baclofen 60 mg/d, benzodiazepam 15 mg/d, and buspirone 15 mg/d moderately ameliorated his symptoms. Three five-day courses of IVIG were given at monthly intervals, during and after which the muscle stiffness and EMG discharges gradually ameliorated. By six weeks after the final IVIG, he had improved his walking speed from half the normal rate to only one-third more than normal. EMG abnormalities had disappeared, and his medications were reduced by one-third. The duration of the remission is not stated.

The authors note that literature reports indicate that IVIG has brought "significant" improvement to six other "stiff man" patients, some of whom had failed to improve with steroids or plasma exchange. —fp