Stiff person syndrome is an uncommon disorder characterized by fluctuating, progressive muscle stiffness, contractions, rigidity, and spasm usually involving the axial muscles.1 Although the cause is unknown, it has been reported to be frequently associated with autoimmune conditions such as diabetes mellitus, pernicious anemia, thyroiditis, and vitiligo. Three different forms of stiff person syndrome are recognized: autoimmune, paraneoplastic, and idiopathic.2
The autoimmune form of the disease accounts for approximately 60% of the cases and is associated with circulating anti-glutamic acid decarboxylase antibodies.3 These antibodies target gamma aminobutyric acidergic neurons and their nerve terminals and are detectable in the serum. The other 40% rely on clinical testing and history for diagnosis4 because there are no consistent characteristic serologic or image abnormalities.
Case Report
A woman (gravida 3 para 2) at 10 weeks’ gestation presented to the triage area with several days of progressive muscle stiffness, body spasms, rigidity, and disabling axial muscle pain.
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— George Amyradakis, MD, S.J. Carlan, MD, Amanpreet Bhullar, MD, Julie Eastwood, RN
This article originally appeared in March 2012 issue of The American Journal of Medicine.
