John Libbey Eurotext

Epileptic Disorders

The Educational Journal of the International League Against Epilepsy

Malformations of cortical development and epilepsies: Volume 11, numéro 3, September 2009

Department of Neuropathology, University Hospital Erlangen, Erlangen, Germany, Daljit S. and Elaine Sarkaria Chair in Diagnostic Medicine, Departments of Pathology, Laboratory Medicine (Neuropathology) and Neurology, UCLA Medical Center, and David Geffen School of Medicine at UCLA, Los Angeles, USA, Texas Children’s Hospital and The Methodist Hospital, Houston, USA, Department of (Neuro)Pathology, Academic Medical Center, University of Amsterdam, and Stichting Epilepsie Instellingen Nederland, Heemstede, The Netherlands, Neuropathology, Institute of Neurology, University College London, UK, Department of Epilepsy Clinic and Experimental Neurophysiology - Fondazione IRCCS Istituto Neurologico “Carlo Besta”, Milano, Italy
  • Mots-clés : epilepsy, development, cortical dysplasia, neuropathology
  • DOI : 10.1684/epd.2009.0261
  • Page(s) : 181-93
  • Année de parution : 2009

Structural brain abnormalities can be increasingly recognized in patients suffering from intractable focal epilepsies using high-resolution imaging techniques. Epilepsy surgery has become a successful treatment option for many of these patients. A broad spectrum of malformations of cortical development (MCD) can be histopathologically identified in resective surgical brain samples. Here, we discuss neuropathological findings and available classification systems in children and adult patients. Particular emphasis will be paid to the classification system for focal cortical dysplasias (FCD), which can be histopathologically distinguished as type I and II. Also mild forms of cortical malformations (mMCD) may be present, including heterotopic neurons in white matter location. However, different cohorts of epilepsy patients may present with similar histopathological findings and clinico-pathological correlations are not always comparable with respect to outcome prediction. We will, therefore, discuss also the difficulties to classify some FCD variants. Notwithstanding, the underlying pathomechanisms in all FCD entities need to be specified. A comprehensive approach taking all currently available data into consideration will be mandatory to further develop our current understanding of FCDs, and to continuously improve our concept for a reliable classification system.