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==Treatment== ==Treatment==
Treatment is mostly ] with muscle relaxants such as ], which lose their effectiveness as the illness progresses. Because many patients with SPS have circulating antibodies to ], an ] cause the disease has been postulated. In the absence of ], ]-controlled ] to determine treatment efficacy, some authorities recommend humane trials of ] therapy, ] or ] ] infusion. Treatment is mostly ] with muscle relaxants such as ], which lose their effectiveness as the illness progresses. Because many patients with SPS have circulating antibodies to ], an ] cause of the disease has been postulated. In the absence of ], ]-controlled ] to determine treatment efficacy, some authorities recommend humane trials of ] therapy, ] or ] ] infusion.


==References== ==References==

Revision as of 23:28, 2 August 2006

Medical condition
Stiff-person syndrome
SpecialtyNeurology Edit this on Wikidata
Frequency8e-05%

Stiff person syndrome (SPS) (or occasionally, stiff-man syndrome) is a rare neurologic disorder of unknown etiology.

History

SPS was first described by Moersch and Woltman at the Mayo Clinic in 1956 .

Symptoms and prognosis

Those with the illness experience progressive, fluctuating tonic contractions of all muscles, particularly the axial musculature. An inability to walk and general paralysis quickly follow; death usually occurs six to twelve months after diagnosis.

Treatment

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 cause of 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.

See also

External links

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