Stiff man syndrome and related conditions.
Identifieur interne : 003967 ( PubMed/Curation ); précédent : 003966; suivant : 003968Stiff man syndrome and related conditions.
Auteurs : Hans-Michael Meinck [Allemagne] ; Philip D. ThompsonSource :
- Movement disorders : official journal of the Movement Disorder Society [ 0885-3185 ] ; 2002.
English descriptors
- KwdEn :
- Adult, Aged, Diagnosis, Differential, Electromyography, Encephalomyelitis (immunology), Encephalomyelitis (physiopathology), Female, Glutamate Decarboxylase (immunology), Humans, Male, Middle Aged, Muscle Rigidity (diagnosis), Muscle Rigidity (immunology), Muscle Rigidity (physiopathology), Muscle, Skeletal (physiopathology), Myoclonus (immunology), Myoclonus (physiopathology), Spasm (immunology), Spasm (physiopathology), Stiff-Person Syndrome (diagnosis), Stiff-Person Syndrome (immunology), Stiff-Person Syndrome (physiopathology), Upper Extremity (physiopathology).
- MESH :
- chemical , immunology : Glutamate Decarboxylase.
- diagnosis : Muscle Rigidity, Stiff-Person Syndrome.
- immunology : Encephalomyelitis, Muscle Rigidity, Myoclonus, Spasm, Stiff-Person Syndrome.
- physiopathology : Encephalomyelitis, Muscle Rigidity, Muscle, Skeletal, Myoclonus, Spasm, Stiff-Person Syndrome, Upper Extremity.
- Adult, Aged, Diagnosis, Differential, Electromyography, Female, Humans, Male, Middle Aged.
Abstract
The stiff man syndrome (SMS) and its variants, focal SMS, stiff limb (or leg) syndrome (SLS), jerking SMS, and progressive encephalomyelitis with rigidity and myoclonus (PERM), appear to occur more frequently than hitherto thought. A characteristic ensemble of symptoms and signs allows a tentative clinical diagnosis. Supportive ancillary findings include (1) the demonstration of continuous muscle activity in trunk and proximal limb muscles despite attempted relaxation, (2) enhanced exteroceptive reflexes, and (3) antibodies to glutamic acid decarboxylase (GAD) in both serum and spinal fluid. Antibodies to GAD are not diagnostic or specific for SMS and the role of these autoantibodies in the pathogenesis of SMS/SLS/PERM is the subject of debate and difficult to reconcile on the basis of our present knowledge. Nevertheless, evidence is emerging to suggest that SMS/SLS/PERM are manifestations of an immune-mediated chronic encephalomyelitis and immunomodulation is an effective therapeutic approach.
DOI: 10.1002/mds.10279
PubMed: 12360534
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Thompson</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="2002">2002</date><idno type="RBID">pubmed:12360534</idno><idno type="pmid">12360534</idno><idno type="doi">10.1002/mds.10279</idno><idno type="wicri:Area/PubMed/Corpus">003967</idno><idno type="wicri:Area/PubMed/Curation">003967</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">Stiff man syndrome and related conditions.</title><author><name sortKey="Meinck, Hans Michael" sort="Meinck, Hans Michael" uniqKey="Meinck H" first="Hans-Michael" last="Meinck">Hans-Michael Meinck</name><affiliation wicri:level="1"><nlm:affiliation>Department of Neurology, Im Neuenheimerfeld, Heidelberg, Germany.</nlm:affiliation><country xml:lang="fr">Allemagne</country><wicri:regionArea>Department of Neurology, Im Neuenheimerfeld, Heidelberg</wicri:regionArea></affiliation></author><author><name sortKey="Thompson, Philip D" sort="Thompson, Philip D" uniqKey="Thompson P" first="Philip D" last="Thompson">Philip D. Thompson</name></author></analytic><series><title level="j">Movement disorders : official journal of the Movement Disorder Society</title><idno type="ISSN">0885-3185</idno><imprint><date when="2002" type="published">2002</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adult</term><term>Aged</term><term>Diagnosis, Differential</term><term>Electromyography</term><term>Encephalomyelitis (immunology)</term><term>Encephalomyelitis (physiopathology)</term><term>Female</term><term>Glutamate Decarboxylase (immunology)</term><term>Humans</term><term>Male</term><term>Middle Aged</term><term>Muscle Rigidity (diagnosis)</term><term>Muscle Rigidity (immunology)</term><term>Muscle Rigidity (physiopathology)</term><term>Muscle, Skeletal (physiopathology)</term><term>Myoclonus (immunology)</term><term>Myoclonus (physiopathology)</term><term>Spasm (immunology)</term><term>Spasm (physiopathology)</term><term>Stiff-Person Syndrome (diagnosis)</term><term>Stiff-Person Syndrome (immunology)</term><term>Stiff-Person Syndrome (physiopathology)</term><term>Upper Extremity (physiopathology)</term></keywords><keywords scheme="MESH" type="chemical" qualifier="immunology" xml:lang="en"><term>Glutamate Decarboxylase</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Muscle Rigidity</term><term>Stiff-Person Syndrome</term></keywords><keywords scheme="MESH" qualifier="immunology" xml:lang="en"><term>Encephalomyelitis</term><term>Muscle Rigidity</term><term>Myoclonus</term><term>Spasm</term><term>Stiff-Person Syndrome</term></keywords><keywords scheme="MESH" qualifier="physiopathology" xml:lang="en"><term>Encephalomyelitis</term><term>Muscle Rigidity</term><term>Muscle, Skeletal</term><term>Myoclonus</term><term>Spasm</term><term>Stiff-Person Syndrome</term><term>Upper Extremity</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adult</term><term>Aged</term><term>Diagnosis, Differential</term><term>Electromyography</term><term>Female</term><term>Humans</term><term>Male</term><term>Middle Aged</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">The stiff man syndrome (SMS) and its variants, focal SMS, stiff limb (or leg) syndrome (SLS), jerking SMS, and progressive encephalomyelitis with rigidity and myoclonus (PERM), appear to occur more frequently than hitherto thought. A characteristic ensemble of symptoms and signs allows a tentative clinical diagnosis. Supportive ancillary findings include (1) the demonstration of continuous muscle activity in trunk and proximal limb muscles despite attempted relaxation, (2) enhanced exteroceptive reflexes, and (3) antibodies to glutamic acid decarboxylase (GAD) in both serum and spinal fluid. Antibodies to GAD are not diagnostic or specific for SMS and the role of these autoantibodies in the pathogenesis of SMS/SLS/PERM is the subject of debate and difficult to reconcile on the basis of our present knowledge. Nevertheless, evidence is emerging to suggest that SMS/SLS/PERM are manifestations of an immune-mediated chronic encephalomyelitis and immunomodulation is an effective therapeutic approach.</div></front></TEI><pubmed><MedlineCitation Owner="NLM" Status="MEDLINE"><PMID Version="1">12360534</PMID><DateCreated><Year>2002</Year><Month>10</Month><Day>02</Day></DateCreated><DateCompleted><Year>2003</Year><Month>01</Month><Day>08</Day></DateCompleted><DateRevised><Year>2006</Year><Month>11</Month><Day>15</Day></DateRevised><Article PubModel="Print"><Journal><ISSN IssnType="Print">0885-3185</ISSN><JournalIssue CitedMedium="Print"><Volume>17</Volume><Issue>5</Issue><PubDate><Year>2002</Year><Month>Sep</Month></PubDate></JournalIssue><Title>Movement disorders : official journal of the Movement Disorder Society</Title><ISOAbbreviation>Mov. Disord.</ISOAbbreviation></Journal><ArticleTitle>Stiff man syndrome and related conditions.</ArticleTitle><Pagination><MedlinePgn>853-66</MedlinePgn></Pagination><Abstract><AbstractText>The stiff man syndrome (SMS) and its variants, focal SMS, stiff limb (or leg) syndrome (SLS), jerking SMS, and progressive encephalomyelitis with rigidity and myoclonus (PERM), appear to occur more frequently than hitherto thought. A characteristic ensemble of symptoms and signs allows a tentative clinical diagnosis. Supportive ancillary findings include (1) the demonstration of continuous muscle activity in trunk and proximal limb muscles despite attempted relaxation, (2) enhanced exteroceptive reflexes, and (3) antibodies to glutamic acid decarboxylase (GAD) in both serum and spinal fluid. Antibodies to GAD are not diagnostic or specific for SMS and the role of these autoantibodies in the pathogenesis of SMS/SLS/PERM is the subject of debate and difficult to reconcile on the basis of our present knowledge. Nevertheless, evidence is emerging to suggest that SMS/SLS/PERM are manifestations of an immune-mediated chronic encephalomyelitis and immunomodulation is an effective therapeutic approach.</AbstractText><CopyrightInformation>Copyright 2002 Movement Disorder Society</CopyrightInformation></Abstract><AuthorList CompleteYN="Y"><Author ValidYN="Y"><LastName>Meinck</LastName><ForeName>Hans-Michael</ForeName><Initials>HM</Initials><AffiliationInfo><Affiliation>Department of Neurology, Im Neuenheimerfeld, Heidelberg, Germany.</Affiliation></AffiliationInfo></Author><Author ValidYN="Y"><LastName>Thompson</LastName><ForeName>Philip D</ForeName><Initials>PD</Initials></Author></AuthorList><Language>eng</Language><PublicationTypeList><PublicationType UI="D016428">Journal Article</PublicationType><PublicationType UI="D013485">Research Support, Non-U.S. Gov't</PublicationType><PublicationType UI="D016454">Review</PublicationType></PublicationTypeList></Article><MedlineJournalInfo><Country>United States</Country><MedlineTA>Mov Disord</MedlineTA><NlmUniqueID>8610688</NlmUniqueID><ISSNLinking>0885-3185</ISSNLinking></MedlineJournalInfo><ChemicalList><Chemical><RegistryNumber>EC 4.1.1.15</RegistryNumber><NameOfSubstance UI="D005968">Glutamate Decarboxylase</NameOfSubstance></Chemical></ChemicalList><CitationSubset>IM</CitationSubset><MeshHeadingList><MeshHeading><DescriptorName MajorTopicYN="N" UI="D000328">Adult</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D000368">Aged</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D003937">Diagnosis, Differential</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D004576">Electromyography</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D004679">Encephalomyelitis</DescriptorName><QualifierName MajorTopicYN="N" UI="Q000276">immunology</QualifierName><QualifierName MajorTopicYN="N" UI="Q000503">physiopathology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D005260">Female</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D005968">Glutamate Decarboxylase</DescriptorName><QualifierName MajorTopicYN="N" UI="Q000276">immunology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D006801">Humans</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D008297">Male</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D008875">Middle Aged</DescriptorName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D009127">Muscle Rigidity</DescriptorName><QualifierName MajorTopicYN="N" UI="Q000175">diagnosis</QualifierName><QualifierName MajorTopicYN="N" UI="Q000276">immunology</QualifierName><QualifierName MajorTopicYN="N" UI="Q000503">physiopathology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D018482">Muscle, Skeletal</DescriptorName><QualifierName MajorTopicYN="N" UI="Q000503">physiopathology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D009207">Myoclonus</DescriptorName><QualifierName MajorTopicYN="N" UI="Q000276">immunology</QualifierName><QualifierName MajorTopicYN="N" UI="Q000503">physiopathology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D013035">Spasm</DescriptorName><QualifierName MajorTopicYN="N" UI="Q000276">immunology</QualifierName><QualifierName MajorTopicYN="N" UI="Q000503">physiopathology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D016750">Stiff-Person Syndrome</DescriptorName><QualifierName MajorTopicYN="N" UI="Q000175">diagnosis</QualifierName><QualifierName MajorTopicYN="N" UI="Q000276">immunology</QualifierName><QualifierName MajorTopicYN="Y" UI="Q000503">physiopathology</QualifierName></MeshHeading><MeshHeading><DescriptorName MajorTopicYN="N" UI="D034941">Upper Extremity</DescriptorName><QualifierName MajorTopicYN="N" UI="Q000503">physiopathology</QualifierName></MeshHeading></MeshHeadingList><NumberOfReferences>114</NumberOfReferences></MedlineCitation><PubmedData><History><PubMedPubDate PubStatus="pubmed"><Year>2002</Year><Month>10</Month><Day>3</Day><Hour>4</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="medline"><Year>2003</Year><Month>1</Month><Day>9</Day><Hour>4</Hour><Minute>0</Minute></PubMedPubDate><PubMedPubDate PubStatus="entrez"><Year>2002</Year><Month>10</Month><Day>3</Day><Hour>4</Hour><Minute>0</Minute></PubMedPubDate></History><PublicationStatus>ppublish</PublicationStatus><ArticleIdList><ArticleId IdType="pubmed">12360534</ArticleId><ArticleId IdType="doi">10.1002/mds.10279</ArticleId></ArticleIdList></PubmedData></pubmed></record>Pour manipuler ce document sous Unix (Dilib)
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