Seizures in juvenile Huntington's disease: Frequency and characterization in a multicenter cohort

• Published in: Movement disorders Vol. 27; no. 14; pp. 1797 - 1800
• Main Authors: Cloud, Leslie J., Rosenblatt, Adam, Margolis, Russel L., Ross, Christopher A., Pillai, Jagan A., Corey-Bloom, Jody, Tully, Hannah M., Bird, Thomas, Panegyres, Peter K., Nichter, Charles A., Higgins Jr, Donald S., Helmers, Sandra L., Factor, Stewart A., Jones, Randi, Testa, Claudia M.
• Format: Journal Article
• Language: English
• Published: Hoboken Wiley Subscription Services, Inc., A Wiley Company 01.12.2012; Wiley; Wiley Subscription Services, Inc
• Subjects: Adolescent; Age Factors; Age of Onset; Anticonvulsants - therapeutic use; Biological and medical sciences; Child; Child, Preschool; Cohort Studies; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Electroencephalography - methods; Female; Humans; Huntington Disease - drug therapy; Huntington Disease - epidemiology; Huntington's disease; juvenile Huntington's disease; Male; Medical sciences; Movement disorders; Neurology; Retrospective Studies; Risk Factors; seizures; Seizures - classification; Seizures - drug therapy; Seizures - epidemiology; Young Adult; Nervous system diseases; Epilepsy; Huntington disease; Huntington's disease; Genetic disease; Cerebral disorder; Convulsion; juvenile Huntington's disease; Central nervous system disease; seizures; Degenerative disease; Neurological disorder; Extrapyramidal syndrome
• ISSN: 0885-3185; 1531-8257
• DOI: 10.1002/mds.25237

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