Reflex
myoclonic epilepsy in infancy: a multicenter clinical study.

Verrotti, A; Matricardi, S; Capovilla, G; D'Egidio, C; Cusmai, R; Romeo, A; Pruna, D; Pavone, P; Cappanera, S; Granata, T; Gobbi, G; Striano, P; Grosso, S; Parisi, P; Franzoni, E; Striano, S; Spalice, A; Marino, R; Vigevano, F; Coppola, Giangennaro

doi:10.1016/j.eplepsyres.2012.07.004

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.

Titolo:	Reflex myoclonic epilepsy in infancy: a multicenter clinical study.
Autori:	Verrotti A Matricardi S Capovilla G D'Egidio C Cusmai R Romeo A Pruna D Pavone P Cappanera S Granata T Gobbi G Striano P Grosso S Parisi P Franzoni E Striano S Spalice A Marino R Vigevano F COPPOLA, Giangennaro mostra contributor esterni nascondi contributor esterni
Data di pubblicazione:	2013
Rivista:	EPILEPSY RESEARCH
Abstract:	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.
Handle:	http://hdl.handle.net/11386/4578466
Appare nelle tipologie:	1.1.2 Articolo su rivista con ISSN

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