Seizures in Juvenile Huntington's Disease: Frequency and Characterization in a Multicenter Cohort
Identifieur interne : 001042 ( Main/Merge ); précédent : 001041; suivant : 001043Seizures 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="Testa, Claudia M" sort="Testa, Claudia M" uniqKey="Testa C" first="Claudia M." last="Testa">Claudia M. Testa</name>
<affiliation wicri:level="2"><inist:fA14 i1="01"><s1>Department of Neurology, Virginia Commonwealth University</s1>
<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>
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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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</country>
<region><li>Californie</li>
<li>Géorgie (États-Unis)</li>
<li>Maryland</li>
<li>Virginie</li>
<li>Washington (État)</li>
<li>État de New York</li>
</region>
<settlement><li>Seattle</li>
</settlement>
<orgName><li>Université de Washington</li>
</orgName>
</list>
<tree><country name="États-Unis"><region name="Virginie"><name sortKey="Cloud, Leslie J" sort="Cloud, Leslie J" uniqKey="Cloud L" first="Leslie J." last="Cloud">Leslie J. Cloud</name>
</region>
<name sortKey="Bird, Thomas" sort="Bird, Thomas" uniqKey="Bird T" first="Thomas" last="Bird">Thomas Bird</name>
<name sortKey="Corey Bloom, Jody" sort="Corey Bloom, Jody" uniqKey="Corey Bloom J" first="Jody" last="Corey-Bloom">Jody Corey-Bloom</name>
<name sortKey="Factor, Stewart A" sort="Factor, Stewart A" uniqKey="Factor S" first="Stewart A." last="Factor">Stewart A. Factor</name>
<name sortKey="Helmers, Sandra L" sort="Helmers, Sandra L" uniqKey="Helmers S" first="Sandra L." last="Helmers">Sandra L. Helmers</name>
<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>
<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>
<name sortKey="Jones, Randi" sort="Jones, Randi" uniqKey="Jones R" first="Randi" last="Jones">Randi Jones</name>
<name sortKey="Margolis, Russel L" sort="Margolis, Russel L" uniqKey="Margolis R" first="Russel L." last="Margolis">Russel L. Margolis</name>
<name sortKey="Nichter, Charles A" sort="Nichter, Charles A" uniqKey="Nichter C" first="Charles A." last="Nichter">Charles A. Nichter</name>
<name sortKey="Pillai, Jagan A" sort="Pillai, Jagan A" uniqKey="Pillai J" first="Jagan A." last="Pillai">Jagan A. Pillai</name>
<name sortKey="Rosenblatt, Adam" sort="Rosenblatt, Adam" uniqKey="Rosenblatt A" first="Adam" last="Rosenblatt">Adam Rosenblatt</name>
<name sortKey="Ross, Christopher A" sort="Ross, Christopher A" uniqKey="Ross C" first="Christopher A." last="Ross">Christopher A. Ross</name>
<name sortKey="Testa, Claudia M" sort="Testa, Claudia M" uniqKey="Testa C" first="Claudia M." last="Testa">Claudia M. Testa</name>
<name sortKey="Tully, Hannah M" sort="Tully, Hannah M" uniqKey="Tully H" first="Hannah M." last="Tully">Hannah M. Tully</name>
</country>
<country name="Australie"><noRegion><name sortKey="Panegyres, Peter K" sort="Panegyres, Peter K" uniqKey="Panegyres P" first="Peter K." last="Panegyres">Peter K. Panegyres</name>
</noRegion>
</country>
</tree>
</affiliations>
</record>
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