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Stiff-Person Syndrome

Published online by Cambridge University Press:  18 September 2015

Mandar S. Jog ,
Colin D. Lambert  and
Anthony E. Lang
Mandar S. Jog
Affiliation:
Division of Neurology, St. Michael’s Hospital, University of Toronto
Colin D. Lambert*
Affiliation:
Division of Neurology, St. Michael’s Hospital, University of Toronto
Anthony E. Lang
Affiliation:
Division of Neurology, The Toronto Hospital, University of Toronto
*
76 McGill Street, Toronto, Ontario, Canada M5B 1H2
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Abstract:

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The stiff-person syndrome is a disorder of persistent, painful muscle contractions predominately affecting the axial musculature. We describe a patient with this disorder and review its pathophysiology. Molecular biologic and immunologic techniques have recently added to the understanding of the mechanism of this disorder. Association with diseases such as diabetes, vitiligo and hypothyroidism have strengthened the auto-immune nature of this syndrome. Auto-antibodies against glutamic acid decarboxylase (GAD), an intraneuronal enzyme, have been implicated in the etiology of this unique disease. Therapeutic intervention with agents such as benzodiazepines that modify central GABAergic activity have demonstrated significant benefit in patients with stiff-person syndrome.

Type
Articles
Information
Canadian Journal of Neurological Sciences , Volume 19 , Issue 3 , August 1992 , pp. 383 - 388
Copyright
Copyright © Canadian Neurological Sciences Federation 1992

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