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 (11-21Hz) with bilateral myoclonic jerks. Hence, the senile myoclonic epilepsy of Genton is an easily recognizable newer epileptic syndrome of the older Down syndrome patient.
Crespel
Arielle
A
Epilepsy Unit, Montpellier, France.
Gonzalez
Victoria
V
Coubes
Philippe
P
Gelisse
Philippe
P
D000544:Alzheimer Disease / Q000150:complications / Q000523:psychology; D003072:Cognition Disorders / Q000209:etiology / Q000523:psychology; D003704:Dementia / Q000150:complications* / Q000523:psychology; D004314:Down Syndrome / Q000150:complications* / Q000523:psychology; D004569:Electroencephalography; D004576:Electromyography; D004831:Epilepsies, Myoclonic / Q000209:etiology*; D020195:Epilepsy, Reflex / Q000503:physiopathology; D005260:Female; D006801:Humans; D007037:Hypothyroidism / Q000150:complications / Q000523:psychology; D008297:Male; D008568:Memory / Q000502:physiology; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000503:physiopathology