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Seizures in juvenile Huntington's disease: Frequency and characterization in a multicenter cohort
Author(s) -
Cloud Leslie J.,
Rosenblatt Adam,
Margolis Russel L.,
Ross Christopher A.,
Pillai Jagan A.,
CoreyBloom Jody,
Tully Hannah M.,
Bird Thomas,
Panegyres Peter K.,
Nichter Charles A.,
Higgins Donald S.,
Helmers Sandra L.,
Factor Stewart A.,
Jones Randi,
Testa Claudia M.
Publication year - 2012
Publication title -
movement disorders
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.352
H-Index - 198
eISSN - 1531-8257
pISSN - 0885-3185
DOI - 10.1002/mds.25237
Subject(s) - juvenile , huntington's disease , cohort , central nervous system disease , medicine , degenerative disease , multicenter study , cohort study , pediatrics , psychiatry , psychology , disease , biology , genetics , randomized controlled trial
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. © 2012 Movement Disorder Society