J Korean Acad Rehabil Med.  2007 Apr;31(2):232-237.

Stiff-man Syndrome: A case report

• Choi HY
• Sung DH

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 asso-ciation 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.

Keyword

Stiff-man syndrome; Autoimmune; Glutamic acid decarboxylase

MeSH Terms

Antibodies
Autoantibodies
Baclofen
Diazepam
Extremities
Fires
gamma-Aminobutyric Acid
Glutamate Decarboxylase
Humans
Muscle Rigidity
Muscles
Noise
Spasm
Stiff-Person Syndrome*
Synaptic Transmission
Antibodies
Autoantibodies
Baclofen
Diazepam
Glutamate Decarboxylase
gamma-Aminobutyric Acid