The stiff-man syndrome and related disorders.

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.

authors

Thompson PD

doi

10.1016/s1353-8020(01)00029-3

subject

Has Abstract

pub_date

2001-10-01 00:00:00

pages

147-53

issue

2

eissn

1353-8020

issn

1873-5126

pii

S1353802001000293

journal_volume

8

pub_type

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