stiff person syndrome

stiff person syndrome

Stiff-arm syndrome

1mo ago
SOURCE  

Description

A 56-year-old woman experienced progressive stiffness and painful spasms in the right arm leading to a frozen arm within 2 years. She had previously developed cerebellar ataxia (anti–glutamic acid decarboxylase [GAD] antibodies, 1,550 nmol/L; age 52 years). Examination showed marked rigidity and decreased movement range of the right arm with generalized hyperreflexia (figure and video). There were no antiamphiphysin or other paraneoplastic antibodies. Search for malignancies was negative. Diabetes was ruled out. The patient benefited from diazepam and IV immunoglobulin. Stiff-person syndrome (SPS) affects predominantly the trunk and lower limbs.1 This case illustrates that the cerebellar ataxia-SPS spectrum of anti-GAD autoimmunity may occur sequentially rather than concurrently, and a stiff arm, a variant of paraneoplastic SPS,2 may rarely be a focal form of anti-GAD-mediated (nonparaneoplastic) SPS. For more, see: http://www.neurology.org/content/85/12/1088.short