Movement Disorders (revue)

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Seizures in Juvenile Huntington's Disease: Frequency and Characterization in a Multicenter Cohort

Identifieur interne : 002D10 ( PascalFrancis/Curation ); précédent : 002D09; suivant : 002D11

Seizures 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 :

RBID : Pascal:13-0063598

Descripteurs français

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.
pA  
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A11 02  1    @1 ROSENBLATT (Adam)
A11 03  1    @1 MARGOLIS (Russel L.)
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A11 05  1    @1 PILLAI (Jagan A.)
A11 06  1    @1 COREY-BLOOM (Jody)
A11 07  1    @1 TULLY (Hannah M.)
A11 08  1    @1 BIRD (Thomas)
A11 09  1    @1 PANEGYRES (Peter K.)
A11 10  1    @1 NICHTER (Charles A.)
A11 11  1    @1 HIGGINS (Donald S. JR)
A11 12  1    @1 HELMERS (Sandra L.)
A11 13  1    @1 FACTOR (Stewart A.)
A11 14  1    @1 JONES (Randi)
A11 15  1    @1 TESTA (Claudia M.)
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C01 01    ENG  @0 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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C03 03  X  FRE  @0 Chorée de Huntington @5 03
C03 03  X  ENG  @0 Huntington disease @5 03
C03 03  X  SPA  @0 Corea Huntington @5 03
C03 04  X  FRE  @0 Pathologie du système nerveux @5 04
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C07 01  X  SPA  @0 Encéfalo patología @5 37
C07 02  X  FRE  @0 Pathologie du système nerveux central @5 38
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C07 02  X  SPA  @0 Sistema nervosio central patología @5 38
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Pascal:13-0063598

Le document en format XML

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<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>
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