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In 1956, Moersch and Woltman1 described 14 patients who presented with extreme muscle spasms and stiffness in the setting of an otherwise normal neurologic examination. These patients showed characteristic axial muscle contractions and exaggerated, painful stimulus-induced spasms. The Moersch-Woltman syndrome, initially called stiff-man syndrome, is now commonly known as stiff-person syndrome (SPS), since a majority of patients are women. A subsequent report2 noted an unusual clinical response to benzodiazepines. Relatively large doses of diazepam were used to suppress the increased muscle activity without causing the expected degree of sedation. Patients with SPS show a characteristic dissociation between the sedative and muscle relaxing effects of these GABAA agonists. Variants of SPS include the stiff-limb syndrome and paraneoplastic encephalomyelitis with rigidity and spasms. The stiff-limb syndrome presents as painful stiffness and spasms limited to one or more limbs without the usual axial muscle involvement of SPS.
Antibodies against glutamic acid decarboxylase (GAD, the enzyme that converts glutamate to gamma-aminobutyric acid [GABA]) are found …
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