Stiff-arm syndrome

摘要: A 56-year-old woman experienced progressive stiffness and painful spasms in the right arm leading to a frozen 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 decreased movement range of with generalized hyperreflexia (figure video). There were no antiamphiphysin or other paraneoplastic antibodies. Search for malignancies was negative. Diabetes ruled out. The patient benefited from diazepam IV immunoglobulin. Stiff-person syndrome (SPS) affects predominantly trunk lower limbs.1 This case illustrates that ataxia-SPS spectrum anti-GAD autoimmunity may occur sequentially rather than concurrently, stiff arm, variant SPS,2 rarely be focal form anti-GAD-mediated (nonparaneoplastic) SPS.