The stiff-man syndrome and related disorders

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dc.contributor.authorThompson, P.
dc.date.issued2001
dc.descriptionCopyright © 2001 Elsevier Science Ltd. All rights reserved.
dc.description.abstractThe 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.
dc.description.statementofresponsibilityP. D. Thompson
dc.identifier.citationParkinsonism and Related Disorders, 2001; 8(2):147-153
dc.identifier.doi10.1016/S1353-8020(01)00029-3
dc.identifier.issn1353-8020
dc.identifier.issn1873-5126
dc.identifier.urihttp://hdl.handle.net/2440/9739
dc.language.isoen
dc.publisherElsevier Sci Ltd
dc.source.urihttps://doi.org/10.1016/s1353-8020(01)00029-3
dc.subjectHumans
dc.subjectMuscle Rigidity
dc.subjectStiff-Person Syndrome
dc.subjectEncephalomyelitis
dc.subjectSpasm
dc.titleThe stiff-man syndrome and related disorders
dc.typeJournal article
pubs.publication-statusPublished

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