Stiff-person syndrome: an autoimmune disease.
Identifieur interne : 002934 ( Ncbi/Checkpoint ); précédent : 002933; suivant : 002935Stiff-person syndrome: an autoimmune disease.
Auteurs : P. Blum [États-Unis] ; Joseph Jankovic [États-Unis]Source :
- Movement disorders : official journal of the Movement Disorder Society [ 0885-3185 ] ; 1991.
English descriptors
- KwdEn :
- Autoantibodies (analysis), Autoimmune Diseases (diagnosis), Autoimmune Diseases (drug therapy), Autoimmune Diseases (immunology), Baclofen (administration & dosage), Diazepam (administration & dosage), Drug Administration Schedule, Drug Therapy, Combination, Female, Glutamate Decarboxylase (immunology), Humans, Male, Middle Aged, Muscle Rigidity (diagnosis), Muscle Rigidity (drug therapy), Muscle Rigidity (immunology), Muscle Tonus (drug effects), Neurologic Examination, Prednisone (administration & dosage).
- MESH :
- chemical , administration & dosage : Baclofen, Diazepam, Prednisone.
- chemical , analysis : Autoantibodies.
- diagnosis : Autoimmune Diseases, Muscle Rigidity.
- drug effects : Muscle Tonus.
- drug therapy : Autoimmune Diseases, Muscle Rigidity.
- immunology : Autoimmune Diseases, Glutamate Decarboxylase, Muscle Rigidity.
- Drug Administration Schedule, Drug Therapy, Combination, Female, Humans, Male, Middle Aged, Neurologic Examination.
Abstract
Stiff-person syndrome (SPS) is characterized by progressive, usually symmetric rigidity of the axial muscles with superimposed painful spasms precipitated by tactile stimuli, passive stretch, volitional movement of affected or unaffected muscles, startling noises, and emotional stimuli. Electromyography demonstrates continuous normal motor unit potentials in the affected muscles. Both the rigidity and the spasms are relieved by sleep, general anesthesia, myoneural blockade, peripheral nerve blockade, and partially by diazepam. Evidence for an autoimmune etiology of SPS includes its association with other autoimmune diseases and autoantibodies and the presence of antibodies against glutamic acid decarboxylase (GAD) in the cerebrospinal fluid (CSF) of many affected patients. We describe two patients with this syndrome who had GAD antibodies in both CSF and serum. Partial relief of the symptoms in these patients by corticosteroid therapy provides additional evidence of an autoimmune etiology of SPS and of the role of immunotherapy in its treatment.
DOI: 10.1002/mds.870060104
PubMed: 2005917
Affiliations:
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Blum</name><affiliation wicri:level="2"><nlm:affiliation>Department of Neurology, Baylor College of Medicine, Houston, Texas 77030.</nlm:affiliation><country xml:lang="fr">États-Unis</country><placeName><region type="state">Texas</region></placeName><wicri:cityArea>Department of Neurology, Baylor College of Medicine, Houston</wicri:cityArea></affiliation></author><author><name sortKey="Jankovic, J" sort="Jankovic, J" uniqKey="Jankovic J" first="J" last="Jankovic">Joseph Jankovic</name><affiliation><country>États-Unis</country><placeName><settlement type="city">Houston</settlement><region type="state">Texas</region></placeName><orgName type="university" n="3">Baylor College of Medicine</orgName></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="1991">1991</date><idno type="RBID">pubmed:2005917</idno><idno type="pmid">2005917</idno><idno type="doi">10.1002/mds.870060104</idno><idno type="wicri:Area/PubMed/Corpus">004E11</idno><idno type="wicri:Area/PubMed/Curation">004E11</idno><idno type="wicri:Area/PubMed/Checkpoint">004D90</idno><idno type="wicri:Area/Ncbi/Merge">002934</idno><idno type="wicri:Area/Ncbi/Curation">002934</idno><idno type="wicri:Area/Ncbi/Checkpoint">002934</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">Stiff-person syndrome: an autoimmune disease.</title><author><name sortKey="Blum, P" sort="Blum, P" uniqKey="Blum P" first="P" last="Blum">P. Blum</name><affiliation wicri:level="2"><nlm:affiliation>Department of Neurology, Baylor College of Medicine, Houston, Texas 77030.</nlm:affiliation><country xml:lang="fr">États-Unis</country><placeName><region type="state">Texas</region></placeName><wicri:cityArea>Department of Neurology, Baylor College of Medicine, Houston</wicri:cityArea></affiliation></author><author><name sortKey="Jankovic, J" sort="Jankovic, J" uniqKey="Jankovic J" first="J" last="Jankovic">Joseph Jankovic</name><affiliation><country>États-Unis</country><placeName><settlement type="city">Houston</settlement><region type="state">Texas</region></placeName><orgName type="university" n="3">Baylor College of Medicine</orgName></affiliation></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="1991" type="published">1991</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Autoantibodies (analysis)</term><term>Autoimmune Diseases (diagnosis)</term><term>Autoimmune Diseases (drug therapy)</term><term>Autoimmune Diseases (immunology)</term><term>Baclofen (administration & dosage)</term><term>Diazepam (administration & dosage)</term><term>Drug Administration Schedule</term><term>Drug Therapy, Combination</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 (drug therapy)</term><term>Muscle Rigidity (immunology)</term><term>Muscle Tonus (drug effects)</term><term>Neurologic Examination</term><term>Prednisone (administration & dosage)</term></keywords><keywords scheme="MESH" type="chemical" qualifier="administration & dosage" xml:lang="en"><term>Baclofen</term><term>Diazepam</term><term>Prednisone</term></keywords><keywords scheme="MESH" type="chemical" qualifier="analysis" xml:lang="en"><term>Autoantibodies</term></keywords><keywords scheme="MESH" qualifier="diagnosis" xml:lang="en"><term>Autoimmune Diseases</term><term>Muscle Rigidity</term></keywords><keywords scheme="MESH" qualifier="drug effects" xml:lang="en"><term>Muscle Tonus</term></keywords><keywords scheme="MESH" qualifier="drug therapy" xml:lang="en"><term>Autoimmune Diseases</term><term>Muscle Rigidity</term></keywords><keywords scheme="MESH" qualifier="immunology" xml:lang="en"><term>Autoimmune Diseases</term><term>Glutamate Decarboxylase</term><term>Muscle Rigidity</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Drug Administration Schedule</term><term>Drug Therapy, Combination</term><term>Female</term><term>Humans</term><term>Male</term><term>Middle Aged</term><term>Neurologic Examination</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Stiff-person syndrome (SPS) is characterized by progressive, usually symmetric rigidity of the axial muscles with superimposed painful spasms precipitated by tactile stimuli, passive stretch, volitional movement of affected or unaffected muscles, startling noises, and emotional stimuli. Electromyography demonstrates continuous normal motor unit potentials in the affected muscles. Both the rigidity and the spasms are relieved by sleep, general anesthesia, myoneural blockade, peripheral nerve blockade, and partially by diazepam. Evidence for an autoimmune etiology of SPS includes its association with other autoimmune diseases and autoantibodies and the presence of antibodies against glutamic acid decarboxylase (GAD) in the cerebrospinal fluid (CSF) of many affected patients. We describe two patients with this syndrome who had GAD antibodies in both CSF and serum. Partial relief of the symptoms in these patients by corticosteroid therapy provides additional evidence of an autoimmune etiology of SPS and of the role of immunotherapy in its treatment.</div></front></TEI><affiliations><list><country><li>États-Unis</li></country><region><li>Texas</li></region><settlement><li>Houston</li></settlement><orgName><li>Baylor College of Medicine</li></orgName></list><tree><country name="États-Unis"><region name="Texas"><name sortKey="Blum, P" sort="Blum, P" uniqKey="Blum P" first="P" last="Blum">P. Blum</name></region><name sortKey="Jankovic, J" sort="Jankovic, J" uniqKey="Jankovic J" first="J" last="Jankovic">Joseph Jankovic</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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