TY - JOUR
T1 - Reflex myoclonic epilepsy in infancy
T2 - A multicenter clinical study
AU - Verrotti, Alberto
AU - Matricardi, Sara
AU - Capovilla, Giuseppe
AU - D'Egidio, Claudia
AU - Cusmai, Raffaella
AU - Romeo, Antonino
AU - Pruna, Dario
AU - Pavone, Piero
AU - Cappanera, Silvia
AU - Granata, Tiziana
AU - Gobbi, Giuseppe
AU - Striano, Pasquale
AU - Grosso, Salvatore
AU - Parisi, Pasquale
AU - Franzoni, Emilio
AU - Striano, Salvatore
AU - Spalice, Alberto
AU - Marino, Raffaella
AU - Vigevano, Federico
AU - Coppola, Giangennaro
PY - 2013/2
Y1 - 2013/2
N2 - 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.
AB - 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.
KW - Acoustic and/or tactile stimuli
KW - Idiopathic generalized epilepsy
KW - Long-term cognitive outcome
KW - Reflex myoclonic epilepsy in infancy
KW - Valproate treatment
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U2 - 10.1016/j.eplepsyres.2012.07.004
DO - 10.1016/j.eplepsyres.2012.07.004
M3 - Article
C2 - 22819072
AN - SCOPUS:84873096487
VL - 103
SP - 237
EP - 244
JO - Epilepsy Research
JF - Epilepsy Research
SN - 0920-1211
IS - 2-3
ER -