title: Reflex myoclonic epilepsy in infancy: a multicenter clinical study.
abstract: OBJECTIVE
  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.
authors: Department of Pediatrics, University of Chieti, Chieti, Italy. averrott@unich.it
authors: Alberto
authors: A
authors: Verrotti
authors: Sara
authors: S
authors: Matricardi
authors: Giuseppe
authors: G
authors: Capovilla
authors: Claudia
authors: C
authors: D'Egidio
authors: Raffaella
authors: R
authors: Cusmai
authors: Antonino
authors: A
authors: Romeo
authors: Dario
authors: D
authors: Pruna
authors: Piero
authors: P
authors: Pavone
authors: Silvia
authors: S
authors: Cappanera
authors: Tiziana
authors: T
authors: Granata
authors: Giuseppe
authors: G
authors: Gobbi
authors: Pasquale
authors: P
authors: Striano
authors: Salvatore
authors: S
authors: Grosso
authors: Pasquale
authors: P
authors: Parisi
authors: Emilio
authors: E
authors: Franzoni
authors: Salvatore
authors: S
authors: Striano
authors: Alberto
authors: A
authors: Spalice
authors: Raffaella
authors: R
authors: Marino
authors: Federico
authors: F
authors: Vigevano
authors: Giangennaro
authors: G
authors: Coppola
mesh_terms: D000161:Acoustic Stimulation / Q000379:methods; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography / Q000379:methods; D004831:Epilepsies, Myoclonic / Q000175:diagnosis* / Q000453:epidemiology* / Q000503:physiopathology; D020195:Epilepsy, Reflex / Q000175:diagnosis* / Q000453:epidemiology* / Q000503:physiopathology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D014110:Touch / Q000502:physiology