Sunday, December 8, 2013

Stiff-Person Syndrome Part 3 of 5

Stiff-Person Syndrome, a very rare condition. So what is known about? Scientists don’t yet understand what causes SPS but research indicates that it is the result of an autoimmune response gone awry in the brain and spinal cord. It may begin very subtly during a period of emotional stress. SPS is often misdiagnosed as Parkinson’s disease, multiple sclerosis, fibromyalgia, psychosomatic illness, or anxiety and phobia. Studies have confirmed that it is typically characterized by a high titer of anti-GAD (glutamic acid decarboxylase) antibodies. While the absence of antibodies in the serum does not rule out SPS, the presence of anti-GAD autoantibodies strongly supports that diagnosis (99% specific by immunocytochemistry). What isn’t known for sure is whether the antibodies have a causative role or are the result of another process. Interestingly, SPS has not been described in members of the same family and there is no known genetic predisposition. Stiff-person syndrome is considered by many researchers to be a spectrum of disease ranging from the involvement of just one area of the body to a widespread, rapidly progressive form that also includes involvement of the brain stem and spinal cord.  Shane, a man I met on a recent airline flight to Australia, who has been diagnosed with SPS, is learning how to manage the pain through his own rehabilitation program. 

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