Benign myoclonic epilepsy in infancy followed by childhood absence epilepsy

Antonina Fontana, Salvatore Mangano, Giuseppa Renata Mangano, Maurizio Montalto, Aldo Barbagallo, Federico Zara

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11 Citations (Scopus)


Benign myoclonic epilepsy in infancy (BMEI) is a rare syndrome included among idiopathic generalizedepilepsies (IGE) and syndromes with age-related onset. Recently, it has been shown that a few patientswith BMEI later had other epilepsy types mainly IGE but never childhood absence epilepsy (CAE).We report a patient who at 11 months of age showed isolated myoclonic jerks occurring several timesa day. The ictal video-EEG and polygraphic recording revealed generalized discharge of spike-wave (SW)lasting 1–2 s associated with isolated bilateral synchronous jerk involving mainly the upper limbscontrolled by valproic acid (VPA).At 6 years and 8 months the child developed a new electroclinical feature recognized as CAE. The ictalEEG disclosed a burst of rhythmic 3 Hz generalized SW.Our case is the first patient with BMEI reported in the literature who later developed a CAE.This finding suggests a common neurobiological and genetic link between different age-relatedepileptic phenotypes.
Original languageEnglish
Pages (from-to)727-730
Number of pages4
Publication statusPublished - 2011

All Science Journal Classification (ASJC) codes

  • Neurology
  • Clinical Neurology


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