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Revision as of 17:38, 30 March 2006
First described by Moersch and Woltman at the Mayo Clinic in 1956, stiff person syndrome (SPS), or occasionally, stiff-man syndrome, is a rare neurologic disorder of unknown etiology. Those with the illness experience progressive, fluctuating tonic contractions of all muscles, particularly the axial musculature. Inability to walk and paralysis quickly ensues; death usually occurs six to twelve months after diagnosis.
Treatment is mostly palliative with muscle relaxants such as benzodiazepines, which lose their effectiveness as the illness progresses. Because many patients with SPS have circulating antibodies to glutamic acid decarboxylase, an autoimmune genesis to the disease has been postulated. In the absence of double-blind, placebo-controlled class A trials to determine treatment efficacy, some authorities recommend humane trials of immunosuppressive therapy, plasmapheresis or intravenous immunoglobulin infusion.
References
- MOERSCH FP, WOLTMAN HW (1956). "Progressive fluctuating muscular rigidity and spasm ("stiff-man" syndrome); report of a case and some observations in 13 other cases". Mayo Clin Proc. 31 (15): 421–7. PMID 13350379.
- Murinson BB (2004). "Stiff-person syndrome". Neurologist. 10 (3): 131–7. PMID 15140273.
External Links
- Neuroimmunology, The Medical School, Birmingham University - Abid R Karim, Birmingham UK