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Type: Journal article
Title: The stiff-man syndrome and related disorders
Author: Thompson, P.
Citation: Parkinsonism and Related Disorders, 2001; 8(2):147-153
Publisher: Elsevier Sci Ltd
Issue Date: 2001
ISSN: 1353-8020
Statement of
P. D. Thompson
Abstract: The stiff-man syndrome (SMS) is characterised by rigidity and spasm of predominantly axial and proximal limb muscles. The cause of the condition is unknown but the finding of antibodies to glutamic acid decarboxylase (GAD) in approximately 60% of patients has suggested an autoimmune basis. Pathological findings are limited to a small number of cases which are reviewed in this paper. In some, evidence of an inflammatory aetiology has been found, and there appears to be overlap with progressive encephalomyelitis with rigidity (PER) which may present with a similar clinical picture. The spontaneous muscle activity in SMS and PER is of central origin, related to release of polysynaptic spinal and brainstem reflexes. The SMS is readily distinguished from the continuous muscle activity, spasm and cramps of Isaac's syndrome and neuromyotonia which originate in the peripheral nervous system. Fasciculations, myokymia, myotonia and complex repetitive discharges are characteristic of these peripheral neuromuscular disorders.
Keywords: Humans
Muscle Rigidity
Stiff-Person Syndrome
Description: Copyright © 2001 Elsevier Science Ltd. All rights reserved.
DOI: 10.1016/S1353-8020(01)00029-3
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