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First described by Moersch and Woltman at the ] in 1956,{{ref|mws}} '''stiff person syndrome''' (SPS), or occasionally, '''stiff-man syndrome''', is a rare ] disorder of unknown ]. Those with the illness experience progressive, fluctuating tonic contractions of all muscles, particularly the axial musculature. Inability to walk and ] quickly ensues; death usually occurs six to twelve months after diagnosis. '''Stiff person syndrome''' (SPS) (or occasionally, '''stiff-man syndrome''') is a rare ] disorder of unknown ]. SPS was first described by Moersch and Woltman at the ] in 1956 {{ref|mws}}.


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 ] genesis to the disease has been postulated. In the absence of ], ]-controlled ] to determine treatment efficacy, some authorities recommend humane trials of ] therapy, ] or intravenous ] infusion. Those with the illness experience progressive, fluctuating tonic contractions of all ]s, particularly the ] musculature. An inability to ] and general ] quickly follow; death usually occurs six to twelve months after diagnosis.
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.


==References== ==References==

Revision as of 14:30, 10 May 2006

Stiff person syndrome (SPS) (or occasionally, stiff-man syndrome) is a rare neurologic disorder of unknown etiology. SPS was first described by Moersch and Woltman at the Mayo Clinic in 1956 .

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 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 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.

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