Stiff-man syndrome is a well-described, but rare and often overlooked,
neuromuscular syndrome of rigidity, spasm, and gait abnormality that
is associated with several endocrinologic and autoimmune disorders. A
patient exhibiting many typical features of stiff-man syndrome had int
ermittent symptoms for 22 years before the correct diagnosis was made.
Similar to many described patients, she was diabetic, hyperthyroid, a
nd had elevated islet cell, antithyroid, and glutamic acid decarboxyla
se antibody levels. The high frequency of diabetes mellitus among pati
ents with stiff-man syndrome is emphasized, as is increasing evidence
to suggest that elaboration of anti-glutamic acid decarboxylase and an
ti-islet cell antibodies may play a role in the pathophysiologic state
of the disorder.