Reflex myoclonic epilepsy in infancy (RMEI) is a rare clinical entity characterized by the occurrence of myoclonic seizures (MS) triggered by unexpected noise or touch. To describe the clinical and electroencephalographic (EEG) features of reflex myoclonic epilepsy in infancy (RMEI) and long-term cognitive outcome, a multicenter study was carried out in order to collect a meaningful number of patients with RMEI. We enrolled 31 children from 16 neuropediatric centers in Italy, who underwent clinical and video-EEG evaluation. Cognitive assessment was performed in all patients using standardized psychometric tests. The age at onset ranged from 3 to 24 months of age. Seizures were characterised in all patients by symmetric myoclonic seizures (MS), triggered by sudden unexpected acoustic (38.7 %) or tactile stimuli (29%) or both (29%). Spontaneous attacks were reported in 32.2% of the cases. Ictal EEG showed generalized high-amplitude 3 Hz polyspike and wave discharges, synchronous with brief rhythmic bursts of electromyographic activity. Patients were re-evaluated after a period of 7.2 ± 5.6 years. The prognosis for seizure control was excellent in all cases and reflex MS disappeared spontaneously or after valproate treatment. The cognitive outcome was excellent in 90.3% of children. RMEI appears to be a variety of idiopathic generalized epilepsy with early onset and specific features that occurs in developmentally normal children.
|Translated title of the contribution||Long-term outcome of reflex myoclonic epilepsy in infancy|
|Number of pages||7|
|Journal||Bollettino - Lega Italiana contro l'Epilessia|
|Publication status||Published - Apr 2013|
ASJC Scopus subject areas
- Clinical Neurology