Senile myoclonic epilepsy of Genton: Two cases in Down syndrome with dementia and late onset epilepsy

Senile myoclonic epilepsy of Genton is a newer epileptic syndrome of the older patients with Down syndrome associated with an Alzheimer-type dementia. We report two observations in whom the clinical and electroencephalographic features are consistent with the description of this syndrome. Both experienced a progressive deterioration of cognitive functions few years before the onset of the epilepsy. The EEG was characteristic with generalized fast spike-waves or polyspikes or polyspike-waves with or without bilateral myoclonic jerks especially at awakenings. One patient had a photoparoxysmal response (11-21 Hz) with bilateral myoclonic jerks. Hence, the senile myoclonic epilepsy of Genton is an easily recognizable newer epileptic syndrome of the older Down syndrome patient. (c) 2007 Elsevier B.V. All rights reserved.