Purpose: To describe the clinical and electroencephalographic (EEG) features of reflex myoclonic epilepsy in infancy (RMEI) and long-term cognitive outcome. Methods: We enrolled 31 children from 16 neuropediatric centres in Italy, who underwent clinical and video-EEG evaluation. Cognitive assessment was performed in all patients using standardized psychometric tests. Results: 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. Conclusions: RMEI appears to be a variety of idiopathic generalized epilepsy with specific features that occurs in developmentally normal children.
Reflex myoclonic epilepsy in infancy. A multicenter clinical study / Alberto, Verrotti; S., Matricardia; Giuseppe, Capovilla; Claudia, D'Egidio; Raffaella, Cusmai; Antonino, Romeo; Dario, Pruna; Piero, Pavone; Silvia, Cappanera; Tiziana, Granata; Giuseppe, Gobbi; Pasquale, Striano; Salvatore, Grosso; Parisi, Pasquale; Emilio, Franzoni; Salvatore, Striano; Raffaella, Marino; Federico, Vigevano; Giangennaro, Coppola; Spalice, Alberto. - In: EPILEPSY RESEARCH. - ISSN 0920-1211. - STAMPA. - 103:2-3(2013), pp. 237-244. (Intervento presentato al convegno 10th European Congress on Epileptology tenutosi a London, ENGLAND nel SEP 30-OCT 04, 2012) [10.1016/j.eplepsyres.2012.07.004].
Reflex myoclonic epilepsy in infancy. A multicenter clinical study
PARISI, PasqualeWriting – Review & Editing
;SPALICE, ALBERTO
2013
Abstract
Purpose: To describe the clinical and electroencephalographic (EEG) features of reflex myoclonic epilepsy in infancy (RMEI) and long-term cognitive outcome. Methods: We enrolled 31 children from 16 neuropediatric centres in Italy, who underwent clinical and video-EEG evaluation. Cognitive assessment was performed in all patients using standardized psychometric tests. Results: 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. Conclusions: RMEI appears to be a variety of idiopathic generalized epilepsy with specific features that occurs in developmentally normal children.File | Dimensione | Formato | |
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