Seizures in Juvenile Huntington's Disease: Frequency and Characterization in a Multicenter Cohort
Identifieur interne : 002D10 ( PascalFrancis/Curation ); précédent : 002D09; suivant : 002D11Seizures in Juvenile Huntington's Disease: Frequency and Characterization in a Multicenter Cohort
Auteurs : Leslie J. Cloud [États-Unis] ; Adam Rosenblatt [États-Unis] ; Russel L. Margolis [États-Unis] ; Christopher A. Ross [États-Unis] ; Jagan A. Pillai [États-Unis] ; Jody Corey-Bloom [États-Unis] ; Hannah M. Tully [États-Unis] ; Thomas Bird [États-Unis] ; Peter K. Panegyres [Australie] ; Charles A. Nichter [États-Unis] ; Donald S. Jr Higgins [États-Unis] ; Sandra L. Helmers [États-Unis] ; Stewart A. Factor [États-Unis] ; Randi Jones [États-Unis] ; Claudia M. Testa [États-Unis]Source :
- Movement disorders [ 0885-3185 ] ; 2012.
Descripteurs français
- Pascal (Inist)
English descriptors
Abstract
Little is known about the epilepsy that often occurs in the juvenile form of Huntington's disease (HD), but is absent from the adult-onset form. The primary aim of this study was to characterize the seizures in juvenile HD (JHD) subjects with regard to frequency, semiology, defining EEG characteristics, and response to antiepileptic agents. A multicenter, retrospective cohort was identified by database query and/or chart review. Data on age of HD onset, primary HD manifestations, number of CAG repeats, the presence or absence of seizures, seizure type(s), antiepileptic drugs used, subjects' response to antiepileptic drugs (AEDs), and EEG results were assembled, where available. Ninety subjects with genetically confirmed JHD were included. Seizures were present in 38% of subjects and were more likely to occur with younger ages of HD onset. Generalized tonic-clonic seizures were the most common seizure type, followed by tonic, myoclonic, and staring spells. Multiple seizure types commonly occurred within the same individual. Data on EEG findings and AED usage are presented. Seizure risk in JHD increases with younger age of HD onset. Our ability to draw firm conclusions about defining EEG characteristics and response to AEDs was limited by the retrospective nature of the study. Future prospective studies are required.
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<sourceDesc><biblStruct><analytic><title xml:lang="en" level="a">Seizures in Juvenile Huntington's Disease: Frequency and Characterization in a Multicenter Cohort</title>
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<author><name sortKey="Rosenblatt, Adam" sort="Rosenblatt, Adam" uniqKey="Rosenblatt A" first="Adam" last="Rosenblatt">Adam Rosenblatt</name>
<affiliation wicri:level="1"><inist:fA14 i1="02"><s1>Department of Psychiatry, Virginia Commonwealth University</s1>
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<author><name sortKey="Panegyres, Peter K" sort="Panegyres, Peter K" uniqKey="Panegyres P" first="Peter K." last="Panegyres">Peter K. Panegyres</name>
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<author><name sortKey="Nichter, Charles A" sort="Nichter, Charles A" uniqKey="Nichter C" first="Charles A." last="Nichter">Charles A. Nichter</name>
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<author><name sortKey="Higgins, Donald S Jr" sort="Higgins, Donald S Jr" uniqKey="Higgins D" first="Donald S. Jr" last="Higgins">Donald S. Jr Higgins</name>
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<author><name sortKey="Helmers, Sandra L" sort="Helmers, Sandra L" uniqKey="Helmers S" first="Sandra L." last="Helmers">Sandra L. Helmers</name>
<affiliation wicri:level="1"><inist:fA14 i1="10"><s1>Department of Neurology, Emory University</s1>
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<author><name sortKey="Factor, Stewart A" sort="Factor, Stewart A" uniqKey="Factor S" first="Stewart A." last="Factor">Stewart A. Factor</name>
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<author><name sortKey="Jones, Randi" sort="Jones, Randi" uniqKey="Jones R" first="Randi" last="Jones">Randi Jones</name>
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<author><name sortKey="Testa, Claudia M" sort="Testa, Claudia M" uniqKey="Testa C" first="Claudia M." last="Testa">Claudia M. Testa</name>
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<s2>Richmond, Virginia</s2>
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<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="2012">2012</date>
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<profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Convulsion</term>
<term>Epilepsy</term>
<term>Huntington disease</term>
<term>Nervous system diseases</term>
</keywords>
<keywords scheme="Pascal" xml:lang="fr"><term>Epilepsie</term>
<term>Convulsion</term>
<term>Chorée de Huntington</term>
<term>Pathologie du système nerveux</term>
</keywords>
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<front><div type="abstract" xml:lang="en">Little is known about the epilepsy that often occurs in the juvenile form of Huntington's disease (HD), but is absent from the adult-onset form. The primary aim of this study was to characterize the seizures in juvenile HD (JHD) subjects with regard to frequency, semiology, defining EEG characteristics, and response to antiepileptic agents. A multicenter, retrospective cohort was identified by database query and/or chart review. Data on age of HD onset, primary HD manifestations, number of CAG repeats, the presence or absence of seizures, seizure type(s), antiepileptic drugs used, subjects' response to antiepileptic drugs (AEDs), and EEG results were assembled, where available. Ninety subjects with genetically confirmed JHD were included. Seizures were present in 38% of subjects and were more likely to occur with younger ages of HD onset. Generalized tonic-clonic seizures were the most common seizure type, followed by tonic, myoclonic, and staring spells. Multiple seizure types commonly occurred within the same individual. Data on EEG findings and AED usage are presented. Seizure risk in JHD increases with younger age of HD onset. Our ability to draw firm conclusions about defining EEG characteristics and response to AEDs was limited by the retrospective nature of the study. Future prospective studies are required.</div>
</front>
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<fA08 i1="01" i2="1" l="ENG"><s1>Seizures in Juvenile Huntington's Disease: Frequency and Characterization in a Multicenter Cohort</s1>
</fA08>
<fA11 i1="01" i2="1"><s1>CLOUD (Leslie J.)</s1>
</fA11>
<fA11 i1="02" i2="1"><s1>ROSENBLATT (Adam)</s1>
</fA11>
<fA11 i1="03" i2="1"><s1>MARGOLIS (Russel L.)</s1>
</fA11>
<fA11 i1="04" i2="1"><s1>ROSS (Christopher A.)</s1>
</fA11>
<fA11 i1="05" i2="1"><s1>PILLAI (Jagan A.)</s1>
</fA11>
<fA11 i1="06" i2="1"><s1>COREY-BLOOM (Jody)</s1>
</fA11>
<fA11 i1="07" i2="1"><s1>TULLY (Hannah M.)</s1>
</fA11>
<fA11 i1="08" i2="1"><s1>BIRD (Thomas)</s1>
</fA11>
<fA11 i1="09" i2="1"><s1>PANEGYRES (Peter K.)</s1>
</fA11>
<fA11 i1="10" i2="1"><s1>NICHTER (Charles A.)</s1>
</fA11>
<fA11 i1="11" i2="1"><s1>HIGGINS (Donald S. JR)</s1>
</fA11>
<fA11 i1="12" i2="1"><s1>HELMERS (Sandra L.)</s1>
</fA11>
<fA11 i1="13" i2="1"><s1>FACTOR (Stewart A.)</s1>
</fA11>
<fA11 i1="14" i2="1"><s1>JONES (Randi)</s1>
</fA11>
<fA11 i1="15" i2="1"><s1>TESTA (Claudia M.)</s1>
</fA11>
<fA14 i1="01"><s1>Department of Neurology, Virginia Commonwealth University</s1>
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<sZ>1 aut.</sZ>
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</fA14>
<fA14 i1="02"><s1>Department of Psychiatry, Virginia Commonwealth University</s1>
<s2>Richmond, Virginia</s2>
<s3>USA</s3>
<sZ>2 aut.</sZ>
</fA14>
<fA14 i1="03"><s1>Division of Neurobiology, Department of Psychiatry, Department of Neurology, and Program in Cellular and Molecular Medicine, Johns Hopkins University School of Medicine</s1>
<s2>Baltimore, Maryland</s2>
<s3>USA</s3>
<sZ>3 aut.</sZ>
<sZ>4 aut.</sZ>
</fA14>
<fA14 i1="04"><s1>Department of Neurosciences, University of California San Diego</s1>
<s2>San Diego, California</s2>
<s3>USA</s3>
<sZ>5 aut.</sZ>
<sZ>6 aut.</sZ>
</fA14>
<fA14 i1="05"><s1>Department of Neurology, University of Washington and Seattle Children's Hospital</s1>
<s2>Seattle, Washington</s2>
<s3>USA</s3>
<sZ>7 aut.</sZ>
</fA14>
<fA14 i1="06"><s1>Department of Neurology and Geriatrics Research Center, VA Puget Sound Health Care System, University of Washington</s1>
<s2>Seattle, Washington</s2>
<s3>USA</s3>
<sZ>8 aut.</sZ>
</fA14>
<fA14 i1="07"><s1>Neurodegenerative Disorders Research Pty Ltd</s1>
<s2>Subiaco, Western Australia</s2>
<s3>AUS</s3>
<sZ>9 aut.</sZ>
</fA14>
<fA14 i1="08"><s1>Department of Neurology, Albany Medical College</s1>
<s2>Albany, New York</s2>
<s3>USA</s3>
<sZ>10 aut.</sZ>
<sZ>11 aut.</sZ>
</fA14>
<fA14 i1="09"><s1>Neurology Service, Samuel Stratton VA Medical Center</s1>
<s2>Albany, New York</s2>
<s3>USA</s3>
<sZ>11 aut.</sZ>
</fA14>
<fA14 i1="10"><s1>Department of Neurology, Emory University</s1>
<s2>Atlanta, Georgia</s2>
<s3>USA</s3>
<sZ>12 aut.</sZ>
<sZ>13 aut.</sZ>
<sZ>14 aut.</sZ>
</fA14>
<fA20><s1>1797-1800</s1>
</fA20>
<fA21><s1>2012</s1>
</fA21>
<fA23 i1="01"><s0>ENG</s0>
</fA23>
<fA43 i1="01"><s1>INIST</s1>
<s2>20953</s2>
<s5>354000503000040180</s5>
</fA43>
<fA44><s0>0000</s0>
<s1>© 2013 INIST-CNRS. All rights reserved.</s1>
</fA44>
<fA45><s0>10 ref.</s0>
</fA45>
<fA47 i1="01" i2="1"><s0>13-0063598</s0>
</fA47>
<fA60><s1>P</s1>
</fA60>
<fA61><s0>A</s0>
</fA61>
<fA64 i1="01" i2="1"><s0>Movement disorders</s0>
</fA64>
<fA66 i1="01"><s0>USA</s0>
</fA66>
<fC01 i1="01" l="ENG"><s0>Little is known about the epilepsy that often occurs in the juvenile form of Huntington's disease (HD), but is absent from the adult-onset form. The primary aim of this study was to characterize the seizures in juvenile HD (JHD) subjects with regard to frequency, semiology, defining EEG characteristics, and response to antiepileptic agents. A multicenter, retrospective cohort was identified by database query and/or chart review. Data on age of HD onset, primary HD manifestations, number of CAG repeats, the presence or absence of seizures, seizure type(s), antiepileptic drugs used, subjects' response to antiepileptic drugs (AEDs), and EEG results were assembled, where available. Ninety subjects with genetically confirmed JHD were included. Seizures were present in 38% of subjects and were more likely to occur with younger ages of HD onset. Generalized tonic-clonic seizures were the most common seizure type, followed by tonic, myoclonic, and staring spells. Multiple seizure types commonly occurred within the same individual. Data on EEG findings and AED usage are presented. Seizure risk in JHD increases with younger age of HD onset. Our ability to draw firm conclusions about defining EEG characteristics and response to AEDs was limited by the retrospective nature of the study. Future prospective studies are required.</s0>
</fC01>
<fC02 i1="01" i2="X"><s0>002B17</s0>
</fC02>
<fC02 i1="02" i2="X"><s0>002B17G</s0>
</fC02>
<fC03 i1="01" i2="X" l="FRE"><s0>Epilepsie</s0>
<s5>01</s5>
</fC03>
<fC03 i1="01" i2="X" l="ENG"><s0>Epilepsy</s0>
<s5>01</s5>
</fC03>
<fC03 i1="01" i2="X" l="SPA"><s0>Epilepsia</s0>
<s5>01</s5>
</fC03>
<fC03 i1="02" i2="X" l="FRE"><s0>Convulsion</s0>
<s5>02</s5>
</fC03>
<fC03 i1="02" i2="X" l="ENG"><s0>Convulsion</s0>
<s5>02</s5>
</fC03>
<fC03 i1="02" i2="X" l="SPA"><s0>Convulsión</s0>
<s5>02</s5>
</fC03>
<fC03 i1="03" i2="X" l="FRE"><s0>Chorée de Huntington</s0>
<s5>03</s5>
</fC03>
<fC03 i1="03" i2="X" l="ENG"><s0>Huntington disease</s0>
<s5>03</s5>
</fC03>
<fC03 i1="03" i2="X" l="SPA"><s0>Corea Huntington</s0>
<s5>03</s5>
</fC03>
<fC03 i1="04" i2="X" l="FRE"><s0>Pathologie du système nerveux</s0>
<s5>04</s5>
</fC03>
<fC03 i1="04" i2="X" l="ENG"><s0>Nervous system diseases</s0>
<s5>04</s5>
</fC03>
<fC03 i1="04" i2="X" l="SPA"><s0>Sistema nervioso patología</s0>
<s5>04</s5>
</fC03>
<fC07 i1="01" i2="X" l="FRE"><s0>Pathologie de l'encéphale</s0>
<s5>37</s5>
</fC07>
<fC07 i1="01" i2="X" l="ENG"><s0>Cerebral disorder</s0>
<s5>37</s5>
</fC07>
<fC07 i1="01" i2="X" l="SPA"><s0>Encéfalo patología</s0>
<s5>37</s5>
</fC07>
<fC07 i1="02" i2="X" l="FRE"><s0>Pathologie du système nerveux central</s0>
<s5>38</s5>
</fC07>
<fC07 i1="02" i2="X" l="ENG"><s0>Central nervous system disease</s0>
<s5>38</s5>
</fC07>
<fC07 i1="02" i2="X" l="SPA"><s0>Sistema nervosio central patología</s0>
<s5>38</s5>
</fC07>
<fC07 i1="03" i2="X" l="FRE"><s0>Trouble neurologique</s0>
<s5>40</s5>
</fC07>
<fC07 i1="03" i2="X" l="ENG"><s0>Neurological disorder</s0>
<s5>40</s5>
</fC07>
<fC07 i1="03" i2="X" l="SPA"><s0>Trastorno neurológico</s0>
<s5>40</s5>
</fC07>
<fC07 i1="04" i2="X" l="FRE"><s0>Syndrome extrapyramidal</s0>
<s5>41</s5>
</fC07>
<fC07 i1="04" i2="X" l="ENG"><s0>Extrapyramidal syndrome</s0>
<s5>41</s5>
</fC07>
<fC07 i1="04" i2="X" l="SPA"><s0>Extrapiramidal síndrome</s0>
<s5>41</s5>
</fC07>
<fC07 i1="05" i2="X" l="FRE"><s0>Maladie dégénérative</s0>
<s5>42</s5>
</fC07>
<fC07 i1="05" i2="X" l="ENG"><s0>Degenerative disease</s0>
<s5>42</s5>
</fC07>
<fC07 i1="05" i2="X" l="SPA"><s0>Enfermedad degenerativa</s0>
<s5>42</s5>
</fC07>
<fC07 i1="06" i2="X" l="FRE"><s0>Maladie héréditaire</s0>
<s5>43</s5>
</fC07>
<fC07 i1="06" i2="X" l="ENG"><s0>Genetic disease</s0>
<s5>43</s5>
</fC07>
<fC07 i1="06" i2="X" l="SPA"><s0>Enfermedad hereditaria</s0>
<s5>43</s5>
</fC07>
<fN21><s1>042</s1>
</fN21>
<fN44 i1="01"><s1>OTO</s1>
</fN44>
<fN82><s1>OTO</s1>
</fN82>
</pA>
</standard>
</inist>
</record>
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