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| ==Treatment== | ==Treatment== | ||
| Treatment is mostly ] with muscle relaxants such as ], which lose their effectiveness as the illness progresses. |
Treatment is mostly ] with muscle relaxants which enhance ] production such as ], which lose their effectiveness as the illness progresses. | ||
| In the absence of ], ]-controlled ] to determine treatment efficacy, some authorities recommend humane trials of ] therapy, ] or ] ] infusion. | |||
| ==References== | ==References== | ||
Revision as of 19:30, 15 February 2007
Medical condition| Stiff-person syndrome | |
|---|---|
| Specialty | Neurology  |
| Frequency | 8e-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.
MRI detection of GABA in the brain have demonstrated reduced levels in stiff-person syndrome.
Treatment
Treatment is mostly palliative with muscle relaxants which enhance GABA production such as benzodiazepines, which lose their effectiveness as the illness progresses. 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
- Neuroimmunology, The Medical School, Birmingham University - A site about the link between GAD and SPS
- stiffperson at NINDS
- Article at clevelandclinic.org
- Article at eNotes