Stiff man syndrome and related conditions
Identifieur interne : 006641 ( Main/Merge ); précédent : 006640; suivant : 006642Stiff man syndrome and related conditions
Auteurs : Hans-Michael Meinck [Allemagne] ; Philip D. Thompson [Australie]Source :
- Movement disorders [ 0885-3185 ] ; 2002.
Descripteurs français
- Pascal (Inist)
- Wicri :
- topic : Homme.
English descriptors
- KwdEn :
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.
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Pascal:02-0583583Le document en format XML
<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en" level="a">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="3"><inist:fA14 i1="01"><s1>Department of Nearology, Im Neuenheimerfeld</s1><s2>Heidelberg</s2><s3>DEU</s3><sZ>1 aut.</sZ></inist:fA14><country>Allemagne</country><placeName><region type="land" nuts="1">Bade-Wurtemberg</region><region type="district" nuts="2">District de Karlsruhe</region><settlement type="city">Heidelberg</settlement></placeName></affiliation></author><author><name sortKey="Thompson, Philip D" sort="Thompson, Philip D" uniqKey="Thompson P" first="Philip D." last="Thompson">Philip D. Thompson</name><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>University Department of Medicine and Department of Neurology, University of Adelaide and Royal Adelaide Hospital</s1><s2>Adelaide, South Australia</s2><s3>AUS</s3><sZ>2 aut.</sZ></inist:fA14><country>Australie</country><wicri:noRegion>Adelaide, South Australia</wicri:noRegion></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">INIST</idno><idno type="inist">02-0583583</idno><date when="2002">2002</date><idno type="stanalyst">PASCAL 02-0583583 INIST</idno><idno type="RBID">Pascal:02-0583583</idno><idno type="wicri:Area/PascalFrancis/Corpus">002635</idno><idno type="wicri:Area/PascalFrancis/Curation">000686</idno><idno type="wicri:Area/PascalFrancis/Checkpoint">002602</idno><idno type="wicri:doubleKey">0885-3185:2002:Meinck H:stiff:man:syndrome</idno><idno type="wicri:Area/Main/Merge">006641</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a">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="3"><inist:fA14 i1="01"><s1>Department of Nearology, Im Neuenheimerfeld</s1><s2>Heidelberg</s2><s3>DEU</s3><sZ>1 aut.</sZ></inist:fA14><country>Allemagne</country><placeName><region type="land" nuts="1">Bade-Wurtemberg</region><region type="district" nuts="2">District de Karlsruhe</region><settlement type="city">Heidelberg</settlement></placeName></affiliation></author><author><name sortKey="Thompson, Philip D" sort="Thompson, Philip D" uniqKey="Thompson P" first="Philip D." last="Thompson">Philip D. Thompson</name><affiliation wicri:level="1"><inist:fA14 i1="02"><s1>University Department of Medicine and Department of Neurology, University of Adelaide and Royal Adelaide Hospital</s1><s2>Adelaide, South Australia</s2><s3>AUS</s3><sZ>2 aut.</sZ></inist:fA14><country>Australie</country><wicri:noRegion>Adelaide, South Australia</wicri:noRegion></affiliation></author></analytic><series><title level="j" type="main">Movement disorders</title><title level="j" type="abbreviated">Mov. disord.</title><idno type="ISSN">0885-3185</idno><imprint><date when="2002">2002</date></imprint></series></biblStruct></sourceDesc><seriesStmt><title level="j" type="main">Movement disorders</title><title level="j" type="abbreviated">Mov. disord.</title><idno type="ISSN">0885-3185</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Differential diagnostic</term><term>Encephalomyelitis</term><term>Human</term><term>Review</term><term>Spasticity</term><term>Stiff man syndrome</term><term>Symptomatology</term></keywords><keywords scheme="Pascal" xml:lang="fr"><term>Homme raide syndrome</term><term>Encéphalomyélite</term><term>Hypertonie spastique</term><term>Article synthèse</term><term>Symptomatologie</term><term>Diagnostic différentiel</term><term>Homme</term></keywords><keywords scheme="Wicri" type="topic" xml:lang="fr"><term>Homme</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><affiliations><list><country><li>Allemagne</li><li>Australie</li></country><region><li>Bade-Wurtemberg</li><li>District de Karlsruhe</li></region><settlement><li>Heidelberg</li></settlement></list><tree><country name="Allemagne"><region name="Bade-Wurtemberg"><name sortKey="Meinck, Hans Michael" sort="Meinck, Hans Michael" uniqKey="Meinck H" first="Hans-Michael" last="Meinck">Hans-Michael Meinck</name></region></country><country name="Australie"><noRegion><name sortKey="Thompson, Philip D" sort="Thompson, Philip D" uniqKey="Thompson P" first="Philip D." last="Thompson">Philip D. Thompson</name></noRegion></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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