Seizures in juvenile Huntington's disease: frequency and characterization in a multicenter cohort.
Identifieur interne : 000C23 ( Main/Exploration ); précédent : 000C22; suivant : 000C24Seizures in juvenile Huntington's disease: frequency and characterization in a multicenter cohort.
Auteurs : Leslie J. Cloud [États-Unis] ; Adam Rosenblatt ; Russel L. Margolis ; Christopher A. Ross ; Jagan A. Pillai ; Jody Corey-Bloom ; Hannah M. Tully ; Thomas Bird ; Peter K. Panegyres ; Charles A. Nichter ; Donald S. Higgins ; Sandra L. Helmers ; Stewart A. Factor ; Randi Jones ; Claudia M. TestaSource :
- Movement disorders : official journal of the Movement Disorder Society [ 1531-8257 ] ; 2012.
English descriptors
- KwdEn :
- Adolescent, Age Factors, Age of Onset, Anticonvulsants (therapeutic use), Child, Child, Preschool, Cohort Studies, Electroencephalography (methods), Female, Humans, Huntington Disease (drug therapy), Huntington Disease (epidemiology), Male, Retrospective Studies, Risk Factors, Seizures (classification), Seizures (drug therapy), Seizures (epidemiology), Young Adult.
- MESH :
- chemical , therapeutic use : Anticonvulsants.
- classification : Seizures.
- drug therapy : Huntington Disease, Seizures.
- epidemiology : Huntington Disease, Seizures.
- methods : Electroencephalography.
- Adolescent, Age Factors, Age of Onset, Child, Child, Preschool, Cohort Studies, Female, Humans, Male, Retrospective Studies, Risk Factors, Young Adult.
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.
DOI: 10.1002/mds.25237
PubMed: 23124580
Affiliations:
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Cloud</name><affiliation wicri:level="1"><nlm:affiliation>Department of Neurology, Virginia Commonwealth University, Richmond, Virginia 23298-0539, USA. lcloud@vcu.edu</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Neurology, Virginia Commonwealth University, Richmond, Virginia 23298-0539</wicri:regionArea><wicri:noRegion>Virginia 23298-0539</wicri:noRegion></affiliation></author><author><name sortKey="Rosenblatt, Adam" sort="Rosenblatt, Adam" uniqKey="Rosenblatt A" first="Adam" last="Rosenblatt">Adam Rosenblatt</name></author><author><name sortKey="Margolis, Russel L" sort="Margolis, Russel L" uniqKey="Margolis R" first="Russel L" last="Margolis">Russel L. Margolis</name></author><author><name sortKey="Ross, Christopher A" sort="Ross, Christopher A" uniqKey="Ross C" first="Christopher A" last="Ross">Christopher A. Ross</name></author><author><name sortKey="Pillai, Jagan A" sort="Pillai, Jagan A" uniqKey="Pillai J" first="Jagan A" last="Pillai">Jagan A. Pillai</name></author><author><name sortKey="Corey Bloom, Jody" sort="Corey Bloom, Jody" uniqKey="Corey Bloom J" first="Jody" last="Corey-Bloom">Jody Corey-Bloom</name></author><author><name sortKey="Tully, Hannah M" sort="Tully, Hannah M" uniqKey="Tully H" first="Hannah M" last="Tully">Hannah M. Tully</name></author><author><name sortKey="Bird, Thomas" sort="Bird, Thomas" uniqKey="Bird T" first="Thomas" last="Bird">Thomas Bird</name></author><author><name sortKey="Panegyres, Peter K" sort="Panegyres, Peter K" uniqKey="Panegyres P" first="Peter K" last="Panegyres">Peter K. Panegyres</name></author><author><name sortKey="Nichter, Charles A" sort="Nichter, Charles A" uniqKey="Nichter C" first="Charles A" last="Nichter">Charles A. Nichter</name></author><author><name sortKey="Higgins, Donald S" sort="Higgins, Donald S" uniqKey="Higgins D" first="Donald S" last="Higgins">Donald S. Higgins</name></author><author><name sortKey="Helmers, Sandra L" sort="Helmers, Sandra L" uniqKey="Helmers S" first="Sandra L" last="Helmers">Sandra L. Helmers</name></author><author><name sortKey="Factor, Stewart A" sort="Factor, Stewart A" uniqKey="Factor S" first="Stewart A" last="Factor">Stewart A. Factor</name></author><author><name sortKey="Jones, Randi" sort="Jones, Randi" uniqKey="Jones R" first="Randi" last="Jones">Randi Jones</name></author><author><name sortKey="Testa, Claudia M" sort="Testa, Claudia M" uniqKey="Testa C" first="Claudia M" last="Testa">Claudia M. Testa</name></author></titleStmt><publicationStmt><idno type="wicri:source">PubMed</idno><date when="2012">2012</date><idno type="doi">10.1002/mds.25237</idno><idno type="RBID">pubmed:23124580</idno><idno type="pmid">23124580</idno><idno type="wicri:Area/PubMed/Corpus">000B81</idno><idno type="wicri:Area/PubMed/Curation">000B81</idno><idno type="wicri:Area/PubMed/Checkpoint">000C39</idno><idno type="wicri:Area/Ncbi/Merge">003881</idno><idno type="wicri:Area/Ncbi/Curation">003881</idno><idno type="wicri:Area/Ncbi/Checkpoint">003881</idno><idno type="wicri:Area/Main/Merge">000C32</idno><idno type="wicri:Area/Main/Curation">000C23</idno><idno type="wicri:Area/Main/Exploration">000C23</idno></publicationStmt><sourceDesc><biblStruct><analytic><title xml:lang="en">Seizures in juvenile Huntington's disease: frequency and characterization in a multicenter cohort.</title><author><name sortKey="Cloud, Leslie J" sort="Cloud, Leslie J" uniqKey="Cloud L" first="Leslie J" last="Cloud">Leslie J. Cloud</name><affiliation wicri:level="1"><nlm:affiliation>Department of Neurology, Virginia Commonwealth University, Richmond, Virginia 23298-0539, USA. lcloud@vcu.edu</nlm:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Neurology, Virginia Commonwealth University, Richmond, Virginia 23298-0539</wicri:regionArea><wicri:noRegion>Virginia 23298-0539</wicri:noRegion></affiliation></author><author><name sortKey="Rosenblatt, Adam" sort="Rosenblatt, Adam" uniqKey="Rosenblatt A" first="Adam" last="Rosenblatt">Adam Rosenblatt</name></author><author><name sortKey="Margolis, Russel L" sort="Margolis, Russel L" uniqKey="Margolis R" first="Russel L" last="Margolis">Russel L. Margolis</name></author><author><name sortKey="Ross, Christopher A" sort="Ross, Christopher A" uniqKey="Ross C" first="Christopher A" last="Ross">Christopher A. Ross</name></author><author><name sortKey="Pillai, Jagan A" sort="Pillai, Jagan A" uniqKey="Pillai J" first="Jagan A" last="Pillai">Jagan A. Pillai</name></author><author><name sortKey="Corey Bloom, Jody" sort="Corey Bloom, Jody" uniqKey="Corey Bloom J" first="Jody" last="Corey-Bloom">Jody Corey-Bloom</name></author><author><name sortKey="Tully, Hannah M" sort="Tully, Hannah M" uniqKey="Tully H" first="Hannah M" last="Tully">Hannah M. Tully</name></author><author><name sortKey="Bird, Thomas" sort="Bird, Thomas" uniqKey="Bird T" first="Thomas" last="Bird">Thomas Bird</name></author><author><name sortKey="Panegyres, Peter K" sort="Panegyres, Peter K" uniqKey="Panegyres P" first="Peter K" last="Panegyres">Peter K. Panegyres</name></author><author><name sortKey="Nichter, Charles A" sort="Nichter, Charles A" uniqKey="Nichter C" first="Charles A" last="Nichter">Charles A. Nichter</name></author><author><name sortKey="Higgins, Donald S" sort="Higgins, Donald S" uniqKey="Higgins D" first="Donald S" last="Higgins">Donald S. Higgins</name></author><author><name sortKey="Helmers, Sandra L" sort="Helmers, Sandra L" uniqKey="Helmers S" first="Sandra L" last="Helmers">Sandra L. Helmers</name></author><author><name sortKey="Factor, Stewart A" sort="Factor, Stewart A" uniqKey="Factor S" first="Stewart A" last="Factor">Stewart A. Factor</name></author><author><name sortKey="Jones, Randi" sort="Jones, Randi" uniqKey="Jones R" first="Randi" last="Jones">Randi Jones</name></author><author><name sortKey="Testa, Claudia M" sort="Testa, Claudia M" uniqKey="Testa C" first="Claudia M" last="Testa">Claudia M. Testa</name></author></analytic><series><title level="j">Movement disorders : official journal of the Movement Disorder Society</title><idno type="eISSN">1531-8257</idno><imprint><date when="2012" type="published">2012</date></imprint></series></biblStruct></sourceDesc></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adolescent</term><term>Age Factors</term><term>Age of Onset</term><term>Anticonvulsants (therapeutic use)</term><term>Child</term><term>Child, Preschool</term><term>Cohort Studies</term><term>Electroencephalography (methods)</term><term>Female</term><term>Humans</term><term>Huntington Disease (drug therapy)</term><term>Huntington Disease (epidemiology)</term><term>Male</term><term>Retrospective Studies</term><term>Risk Factors</term><term>Seizures (classification)</term><term>Seizures (drug therapy)</term><term>Seizures (epidemiology)</term><term>Young Adult</term></keywords><keywords scheme="MESH" type="chemical" qualifier="therapeutic use" xml:lang="en"><term>Anticonvulsants</term></keywords><keywords scheme="MESH" qualifier="classification" xml:lang="en"><term>Seizures</term></keywords><keywords scheme="MESH" qualifier="drug therapy" xml:lang="en"><term>Huntington Disease</term><term>Seizures</term></keywords><keywords scheme="MESH" qualifier="epidemiology" xml:lang="en"><term>Huntington Disease</term><term>Seizures</term></keywords><keywords scheme="MESH" qualifier="methods" xml:lang="en"><term>Electroencephalography</term></keywords><keywords scheme="MESH" xml:lang="en"><term>Adolescent</term><term>Age Factors</term><term>Age of Onset</term><term>Child</term><term>Child, Preschool</term><term>Cohort Studies</term><term>Female</term><term>Humans</term><term>Male</term><term>Retrospective Studies</term><term>Risk Factors</term><term>Young Adult</term></keywords></textClass></profileDesc></teiHeader><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></TEI><affiliations><list><country><li>États-Unis</li></country></list><tree><noCountry><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" sort="Higgins, Donald S" uniqKey="Higgins D" first="Donald S" last="Higgins">Donald S. 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="Panegyres, Peter K" sort="Panegyres, Peter K" uniqKey="Panegyres P" first="Peter K" last="Panegyres">Peter K. Panegyres</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></noCountry><country name="États-Unis"><noRegion><name sortKey="Cloud, Leslie J" sort="Cloud, Leslie J" uniqKey="Cloud L" first="Leslie J" last="Cloud">Leslie J. Cloud</name></noRegion></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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