Stiff man syndrome and related conditions.
Identifieur interne : 000884 ( Ncbi/Checkpoint ); précédent : 000883; suivant : 000885Stiff 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
Affiliations:
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<record><TEI><teiHeader><fileDesc><titleStmt><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="3"><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><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></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><idno type="wicri:Area/PubMed/Checkpoint">003927</idno><idno type="wicri:Area/Ncbi/Merge">000884</idno><idno type="wicri:Area/Ncbi/Curation">000884</idno><idno type="wicri:Area/Ncbi/Checkpoint">000884</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="3"><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><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></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><affiliations><list><country><li>Allemagne</li></country><region><li>Bade-Wurtemberg</li><li>District de Karlsruhe</li></region><settlement><li>Heidelberg</li></settlement></list><tree><noCountry><name sortKey="Thompson, Philip D" sort="Thompson, Philip D" uniqKey="Thompson P" first="Philip D" last="Thompson">Philip D. Thompson</name></noCountry><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></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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