On Dec. 27, 2001, The New England Journal of Medicine reported that intravenous immunoglobulin (IVIg) has brought dramatic relief from symptoms to patients with the rare disorder known as Stiff Person Syndrome (SPS). The authors believe their research supports the theory that SPS results from an autoimmune response gone awry in the brain and spinal cord of the person with this ailment.
SPS is an extremely rare neurological disorder characterized by progressive stiffness and spasms of certain muscles in the trunk and limbs. It sometimes begins with intermittent episodes, often precipitated by surprise or minor physical contact. The incidence of Stiff Person Syndrome (formerly known as Stiffman's Disease) has been estimated at one in a million.
Although the cause of SPS is not known, people with SPS have elevated antibodies in the blood against a particular enzyme, glutamic acid decarboxylase (GAD65). This enzyme is involved in the synthesis of a neurotransmitter that controls muscle movement.
For the study reported in The New England Journal of Medicine, a research team led by Marinos C. Dalakas, M.D., chief of the Neuromuscular Diseases Section of the National Institute of Neurological Disorders and Stoke (NINDS), selected 16 patients who tested positive for the antibodies. For three months, some received IVIg and some received a placebo. One month later, the patients were switched to the alternative therapy. IVIg is an immune-globulin that boosts the immune system and is ordinarily used by people with certain immune deficiencies.
The results of the experiment showed dramatic improvement in symptoms for people with Stiff Person Syndrome who received the IVIg therapy. Eleven of the 14 who finished the study became less stiff and were able to move around significantly better while under treatment.
Until now, the standard treatment for SPS has been diazepam, but Dr. Dalakas said many patients require such high doses of the drug that they become overly sedated. IVIg treats symptoms more successfully and does not have disabling side-effects, according to the researcher.
Additional research is needed on the use of this therapy for Stiff Person Syndrome, and to determine why the autoimmune process seems to go awry in people with this disease. Unfortunately, there are repeated shortages of the immune globulins in the united States, and the biologic treatment is very expensive.
NINDS is part of the National Institutes of Health in Bethesda, Md. The Bayer Corporation and Crescent Health donated IVIg used in the study.
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