Stiff-man Syndrome: A case report.

, Choi, Ha Young; , Sung, Duk Hyun

Case Report

Journal of the Korean Academy of Rehabilitation Medicine 2007;31(2):232-237.

Department of Physical Medicine and Rehabilitation, Samsung Medical Center, Sungkyunkwan University School of Medicine, Korea. dhsung@smc.samsung.co.kr

근육강직 증후군 ⁣증례 보고⁣

최하영, 성덕현

성균관대학교 의과대학 재활의학교실

Abstract

The stiff-man syndrome is a rare and disabling disorder, characterized by muscle rigidity with superimposed painful spasms involving axial and limb musculature. The clinical symptoms are continuous contraction of agonist and antagonist muscles caused by involuntary motor-unit firing at rest and spasms precipitated by tactile stimuli, passive stretch, volitional movement of muscles, startling noises and emotional stimuli. The cause of stiff-man syndrome is unknown but an autoimmune pathogenesis is suspected. The presence of antibodies against glutamic acid decarboxylase, the association of the disease with other autoimmune disorders, and the presence of various autoantibodies contribute to the assumption. The stiff-man syndrome is clinically elusive, but potentially treatable and should be considered in patients with unexplained stiffness and spasms. Drugs that enhance GABA neurotransmission, such as diazepam and baclofen, provide modest relief of clinical symptoms. We described three patients with clinical and electrophysiologic feature of stiff-man syndrome. (J Korean Acad Rehab Med 2007; 31: 232-237)

Key Words: Stiff-man syndrome, Autoimmune, Glutamic acid decarboxylase