title: Benign myoclonic epilepsy in infancy (BMEI): a longitudinal electroclinical study of 22 cases.
abstract: OBJECTIVE
  Benign myoclonic epilepsy in infancy (BMEI) is a nosologically well-defined entity, characterized by myoclonic seizures (MS) in normal children younger than 3 years and by a good long term prognosis. In some cases the seizures are reflex. We studied 22 cases to better define the electroclinical semeiology and evolution of the disorder.
  
  
  METHODS
  Serial electroclinical and neuropsychological assessments, both during wakefulness and during sleep, were performed in 22 otherwise healthy children with spontaneous (17) or reflex (5) MS, recorded by video-EEG-polygraphy since clinical onset.
  
  
  RESULTS
  Seizure onset was between 3 months and 4 years 10 months (50% during first year, 86% before the third year); in reflex cases onset, was earlier than the 14th month. MS recurred during wakefulness and slow sleep in all cases and during REM sleep in reflex cases. MS and related EEG discharges were synchronous or asynchronous. Often ictal EEG discharges were limited to the rolandic and vertex regions (falsely focal paroxysms). Several seizures were subtle and could have escaped recognition. Unusually frequent sleep startles were recorded mostly in reflex cases. MS were well controlled by treatment. At follow-up, between ages 3 and 19 years, four patients had occasional seizures; two had cognitive impairment and three had learning difficulties. No other seizures or cognitive deficits were observed in reflex cases.
  
  
  CONCLUSIONS
  Seizures associated with BMEI are rarely truly generalized and are often so subtle and related to falsely focal paroxysms that their frequency can be underestimated. The reflex form is a well-defined variant with an early onset, peculiar electroclinical features, and a good prognosis.
authors.affiliation: Unit of Child Neuropsychiatry, University of Verona, Verona, Italy.
authors.forename: Francesca
authors.initials: F
authors.lastname: Darra
authors.forename: Elena
authors.initials: E
authors.lastname: Fiorini
authors.forename: Leonardo
authors.initials: L
authors.lastname: Zoccante
authors.forename: Laura
authors.initials: L
authors.lastname: Mastella
authors.forename: Claudia
authors.initials: C
authors.lastname: Torniero
authors.forename: Samuele
authors.initials: S
authors.lastname: Cortese
authors.forename: Lisa
authors.initials: L
authors.lastname: Meneghello
authors.forename: Elena
authors.initials: E
authors.lastname: Fontana
authors.forename: Bernardo Dalla
authors.initials: BD
authors.lastname: Bernardina
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D020820:Dyskinesias / Q000175:diagnosis; D004569:Electroencephalography / Q000706:statistics & numerical data*; D004831:Epilepsies, Myoclonic / Q000175:diagnosis*; D020936:Epilepsy, Benign Neonatal / Q000175:diagnosis; D020195:Epilepsy, Reflex / Q000175:diagnosis; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D009483:Neuropsychological Tests; D011379:Prognosis; D012890:Sleep / Q000502:physiology; D014743:Videotape Recording; D014851:Wakefulness / Q000502:physiology