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 3Hz 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 / A., V., S., M., G., C., C., D., R., C., A., R., D., P., P., P., S., C., T., G., G., G., Striano, P., S., G., P., P., E., F., Striano, S., A., S., R., M., F., V., G., C.. - In: EPILEPSY RESEARCH. - ISSN 0920-1211. - (2012). [10.1016/j.eplepsyres.2012.07.004]
Reflex myoclonic epilepsy in infancy: A multicenter clinical study.
STRIANO, PASQUALE;STRIANO, SALVATORE;
2012
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 3Hz 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.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
