Dystonia, athetosis, and epilepsia partialis continua in a patient with late-onset Rasmussen's encephalitis
Identifieur interne : 002784 ( PascalFrancis/Checkpoint ); précédent : 002783; suivant : 002785Dystonia, athetosis, and epilepsia partialis continua in a patient with late-onset Rasmussen's encephalitis
Auteurs : Steven Frucht [États-Unis]Source :
- Movement disorders [ 0885-3185 ] ; 2002.
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- Pascal (Inist)
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Abstract
Rasmussen's encephalitis is a rare autoimmune disorder characterized by intractable epilepsy and progressive hemispheric dysfunction. The disorder usually affects children, although cases have been reported with symptom onset in late adolescence or adulthood. Myoclonus is common in Rasmussen patients, usually occurring as part of epilepsia partialis continua (EPC); however, other hyperkinetic movements are rare. This report documents a 19-year-old woman with Rasmussen's encephalitis whose clinical presentation was dominated by foot dystonia, arm athetosis, and EPC. Intravenous immunoglobulin improved both hyperkinetic movements and EPC, but benefit was transient. The clinical significance and implications of these findings are discussed.
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<record><TEI><teiHeader><fileDesc><titleStmt><title xml:lang="en" level="a">Dystonia, athetosis, and epilepsia partialis continua in a patient with late-onset Rasmussen's encephalitis</title><author><name sortKey="Frucht, Steven" sort="Frucht, Steven" uniqKey="Frucht S" first="Steven" last="Frucht">Steven Frucht</name><affiliation wicri:level="2"><inist:fA14 i1="01"><s1>Columbia-Presbyterian Medical Center, The Neurological Institute</s1><s2>New York, New York</s2><s3>USA</s3><sZ>1 aut.</sZ></inist:fA14><country>États-Unis</country><placeName><region type="state">État de New York</region></placeName></affiliation></author></titleStmt><publicationStmt><idno type="wicri:source">INIST</idno><idno type="inist">02-0369782</idno><date when="2002">2002</date><idno type="stanalyst">PASCAL 02-0369782 INIST</idno><idno type="RBID">Pascal:02-0369782</idno><idno type="wicri:Area/PascalFrancis/Corpus">002726</idno><idno type="wicri:Area/PascalFrancis/Curation">000595</idno><idno type="wicri:Area/PascalFrancis/Checkpoint">002784</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en" level="a">Dystonia, athetosis, and epilepsia partialis continua in a patient with late-onset Rasmussen's encephalitis</title><author><name sortKey="Frucht, Steven" sort="Frucht, Steven" uniqKey="Frucht S" first="Steven" last="Frucht">Steven Frucht</name><affiliation wicri:level="2"><inist:fA14 i1="01"><s1>Columbia-Presbyterian Medical Center, The Neurological Institute</s1><s2>New York, New York</s2><s3>USA</s3><sZ>1 aut.</sZ></inist:fA14><country>États-Unis</country><placeName><region type="state">État de New York</region></placeName></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>Age of onset</term><term>Athetosis</term><term>Case study</term><term>Continuous</term><term>Diagnosis</term><term>Dystonia</term><term>Epilepsy</term><term>Female</term><term>Partial</term><term>Rasmussen syndrome</term><term>Young adult</term></keywords><keywords scheme="Pascal" xml:lang="fr"><term>Rasmussen syndrome</term><term>Dystonie</term><term>Athétose</term><term>Epilepsie</term><term>Partiel</term><term>Continu</term><term>Age apparition</term><term>Etude cas</term><term>Diagnostic</term><term>Adulte jeune</term><term>Femelle</term></keywords></textClass></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">Rasmussen's encephalitis is a rare autoimmune disorder characterized by intractable epilepsy and progressive hemispheric dysfunction. The disorder usually affects children, although cases have been reported with symptom onset in late adolescence or adulthood. Myoclonus is common in Rasmussen patients, usually occurring as part of epilepsia partialis continua (EPC); however, other hyperkinetic movements are rare. This report documents a 19-year-old woman with Rasmussen's encephalitis whose clinical presentation was dominated by foot dystonia, arm athetosis, and EPC. Intravenous immunoglobulin improved both hyperkinetic movements and EPC, but benefit was transient. 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