Linked Life Data

19349711

Source:http://linkedlifedata.com/resource/pubmed/id/19349711

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:dateCreated
2009-4-7
pubmed:abstractText
Stiff person syndrome (SPS), stiff limb syndrome, jerking SPS and progressive encephalomyelitis with rigidity and myoclonus (PERM) are a family of rare, insidiously progressive diseases of the central nervous system. They all share the core clinical features of appendicular and axial rigidity caused by continuous involuntary motor unit activity, and superimposed stimulus-sensitive spasms. There is good evidence for a primary auto-immune aetiology. Anti-glutamic acid decarboxylase (anti-GAD) antibodies, specifically to the GAD65 isoform, are present in serum or cerebrospinal fluid of 60-80% of patients with SPS and its variants. A paraneoplastic form of SPS is recognized in about 5%, associated with a different profile of auto-antibodies. Repeated intravenous immunoglobulin is the mainstay of disease-modifying therapy in SPS. Rigidity and spasms may be treated symptomatically with benzodiazepines, baclofen, tiagabine and levetiracetam. After an initial progressive phase, patients with SPS generally stabilize over a period of months to years. However, 10% will require prolongedadmission to intensive care at some stage during the disease. Sudden death has been reported in asmany as 10% of patients because of unexplained metabolic acidosis or autonomic crises. The prognosis in paraneoplastic SPS, jerking SPS and PERM, in terms of mortality, is generally worse than in primary SPS.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:issn
1660-4431
pubmed:author
pubmed:copyrightInfo
Copyright (c) 2009 S. Karger AG, Basel.
pubmed:issnType
Print
pubmed:volume
26
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
147-65
pubmed:meshHeading
pubmed:year
2009
pubmed:articleTitle
Stiff person syndrome.
pubmed:affiliation
Institute of Neuroscience, University of Newcastle upon Tyne, Newcastle upon Tyne, UK.
pubmed:publicationType
Journal Article, Review