Atypical "benign" partial epilepsy of childhood or pseudo-lennox syndrome. Part II: family study

Neuropediatrics
H DooseU Stephani

Abstract

Atypical benign partial epilepsy of childhood (ABPE = Pseudo-Lennox syndrome) shows semiologic parallels to Lennox-Gastaut syndrome, however--besides the lack of tonic seizures--it has an entirely different etiology and prognosis. Recently Hahn et al [17] investigated the long-term evolution of 43 cases with ABPE. Symptomatology, EEG findings, and course were found to overlap with Rolandic epilepsy, Landau-Kleffner syndrome and ESES. The incidence of seizures in relatives was determined in the whole series investigated by Hahn et al [17]. Five of 56 siblings suffered from seizures (3 Rolandic seizures; one febrile convulsions; one unclassified). Three fathers reported grand mal. In 29 families of the series of Hahn et al EEG recordings were performed: 22 brothers, 19 sisters and 16 pairs of parents. In 29% of the siblings a sharp wave focus was demonstrable. The rate rose to 40% when only siblings investigated at the age of maximum expression (3 to 10 years) were considered. Sharp wave foci were mostly multifocal and indistinguishable from those observed in siblings of children with Rolandic epilepsy. Photoparoxysmal response and generalized spikes and waves during rest and hyperventilation were also found to be significantly e...Continue Reading

Citations

Aug 23, 2008·Brain : a Journal of Neurology·Chrysostomos P PanayiotopoulosMichael Koutroumanidis
Mar 15, 2006·Epilepsia·Lata VadlamudiSamuel F Berkovic
Oct 24, 2006·Epilepsia·Xavier De TiègePatrick Van Bogaert
Sep 17, 2013·PloS One·Dennis LalBernd A Neubauer
Jun 19, 2014·Human Molecular Genetics·Eva M ReinthalerBernd A Neubauer
Jun 14, 2013·Epilepsia·Friederike MoellerMichael Siniatchkin
May 24, 2012·Neuroscience and Biobehavioral Reviews·Radwa A B BadawyAnthony S Harvey
Nov 26, 2010·Pediatric Neurology·Akiko FujiiMakiko Osawa
Jan 9, 2013·Epilepsia·Meng-Han TsaiIngrid E Scheffer
Mar 5, 2014·Annals of Neurology·Dennis LalBernd A Neubauer
Mar 7, 2014·European Journal of Paediatric Neurology : EJPN : Official Journal of the European Paediatric Neurology Society·Natia JaparidzeUlrich Stephani
Mar 6, 2008·Clinical EEG and Neuroscience·John R Hughes

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