John Libbey Eurotext

EEG characteristics in juvenile Huntington‘s disease: a case report and review of the literature Volume 5, numéro 3, September 2003

Neurophysiology Section and Movement Disorder Section Walter Reed Army Medical Center*, USA Department of Neurology

The clinical features of Juvenile Huntington‘s Disease (J‐HD) differ from those of the more common adult‐onset form, and include cognitive decline, parkinsonism, myoclonus and seizures. A paucity of literature is available describing the electroencephalographic (EEG) findings. We describe the clinical and EEG characteristics of a patient with genetically confirmed J‐HD. A review of previously published cases yielded EEG descriptions in only 23 patients whose disease onset was prior to the age of 32, and only 14 of these were prior to the age of 20. Epileptiform abnormalities were noted in 17 (74%), which was considerably more common than in the adult form. Generalized discharges were noted in nine, with six having polyspike and wave. The remainder had focal or multifocal epileptiform discharges. With genetic testing now available, refinement of the EEG data will be possible.