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Stiff-person syndrome

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First described by Moersch and Woltman at the Mayo Clinic in 1956, stiff person syndrome (SPS) 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 and some authorities recommend humane trials of immunosuppressive therapy, plasmapheresis or intravenous immunoglobulin infusion owing to the absence of double-blind, placebo-controlled class A trials on treatment efficacy.

References

Template:Journal reference issue PMID 13350379


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