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

Epilepsy Res. 2007 Dec;77(2-3):165-8. doi: 10.1016/j.eplepsyres.2007.09.002. Epub 2007 Oct 24.

Abstract

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.

Publication types

  • Case Reports

MeSH terms

  • Alzheimer Disease / complications
  • Alzheimer Disease / psychology
  • Cognition Disorders / etiology
  • Cognition Disorders / psychology
  • Dementia / complications*
  • Dementia / psychology
  • Down Syndrome / complications*
  • Down Syndrome / psychology
  • Electroencephalography
  • Electromyography
  • Epilepsies, Myoclonic / etiology*
  • Epilepsy, Reflex / physiopathology
  • Female
  • Humans
  • Hypothyroidism / complications
  • Hypothyroidism / psychology
  • Male
  • Memory / physiology
  • Middle Aged
  • Muscle, Skeletal / physiopathology