Abstracts

Benign myoclonic epilepsy in infants (BMEI) is a rare epileptic syndrome characterized by only generalized myoclonic seizures in normal children during the first two years. Only few data describe the long-term follow-up. We included the patients with BMEI confirmed by electroencephalogram (EEG) between 1981 and 2002 in four neuropediatric units in France. Clinical and electroencephalographic findings at diagnosis and during the follow-up were collected. Vineland scale and/or Wechsler scale were used to performed neuropsychological evaluation We report 34 patients with BMEI. A family history of febrile seizures (FS) or epilepsy was noted in six patients. A personal history of FS was noted in 11 patients. Eleven patients presented reflex myoclonic seizures. Monotherapy with valproate acid was effective in 23 of the 30 treated patients. Epileptic evolution was known in all patients. Four patients presented seizures after the initial symptoms. Two patients developed a juvenile myoclonic epilepsy and one a cryptogenic partial epilepsy. Neuropsychological outcome was evaluated in 20 patients (10 with Wechsler scales and 17 with Vineland scale) . Cognitive functions were normal in 17 patients. Mental retardation was observed in 3 patients The positive family history of FS or epilepsy suggests that the genetic factor is important in the pathogenesis of BMEI. Reflex myoclonic seizures were frequently observed suggesting that the distinction of two distinctive syndromes is not necessary. Valproate monotherapy was effective. BMEI may be followed by juvenile myoclonic epilepsy. Despite a general favourable neuropsychological outcome, mental retardation can be observed.