INTRODUCTION
Stiff-man Syndrome is a rare disease of the nervous system. Progressively severe muscle stiffness typically develops in the spine and lower extremities; often beginning very subtly during a period of emotional stress. Most patients experience painful episodic muscle spasms that are triggered by sudden stimuli. An auto-immune component is typical and patients often have other auto-immune disorders. Symptoms usually begin in the mid-forties. The severe muscle stiff-ness of SPS responds to benzodiazepines. Testing with an EMG (electro-myogram) needle will assist the diagnosis and testing the blood for anti-GAD (glutamic acid decarboxylase) antibodies (if present at very high levels) will confirm the diagnosis in the majority of people with SPS.

SPS is extremely rare. Although it is not possible to determine the exact prevalence, it may occur in fewer that 1 per million. The disease is more common in women (the ratio is 2 women for every man effected). There is no predilection for any race or ethnic group. There is an association with diabetes and perhaps over half of patients with SPS have or will develop diabetes. Other autoimmune diseases have been found in association with SPS, for example: thyroid disease and vitiligo. There is an increased incidence of epilepsy. An important but especially rare variant of SPS is associated with breast or lung cancer. Characterized by the production of antibodies to amphiphysin, this variant of SPS has certain features that distinguish it from the more typical SPS with GAD antibodies.


         
The Richard T. Johnson
Division of Neuroimmunology & Infectious Diseases
The Johns Hopkins Hospital, Department of Neurology
600 N. Wolfe Street, Baltimore, MD 21287
443-287-4656 (tel)   410-502-8075 (fax)