Till Hartlieb1, Peter Winkler2, Roland Coras3, Tom Pieper2, H Holthausen2, Ingmar Blümcke3, Martin Staudt4, Manfred Kudernatsch5. 1. Hospital for Neuropediatrics and Neurological Rehabilitation, Epilepsy Center for Children and Adolescents, Schön Klinik Vogtareuth, Germany; Paracelsus Medical University, Salzburg, Austria. Electronic address: thartlieb@schoen-kliniken.de. 2. Hospital for Neuropediatrics and Neurological Rehabilitation, Epilepsy Center for Children and Adolescents, Schön Klinik Vogtareuth, Germany. 3. Department of Neuropathology, University Hospital Erlangen, Germany. 4. Hospital for Neuropediatrics and Neurological Rehabilitation, Epilepsy Center for Children and Adolescents, Schön Klinik Vogtareuth, Germany; Department for Pediatric Neurology and Developmental Medicine, University Children Hospital Tübingen, Germany. 5. Paracelsus Medical University, Salzburg, Austria; Epilepsy Center and Department of Neurosurgery, Schön Klinik Vogtareuth, Germany.
Abstract
BACKGROUND: Mild malformation of cortical development with oligodendroglial hyperplasia and epilepsy (MOGHE) is a newly described, rare histopathologic entity detected in resected brain tissue of patients with refractory epilepsies. It shows a predominantly frontal localization causing a difficult-to-treat epilepsy with onset usually in early childhood. Histologically, MOGHE is characterized by blurred gray-white-matter boundaries with increased numbers of heterotopic neurons in the subcortical white matter and increased density of oligodendroglia. Little is known, to date, about radiologic features of MOGHE. Here, we report typical and age-related magnetic resonance (MR) characteristics of MOGHE. PATIENTS AND METHODS: Retrospective analysis of 40 preoperative MR images of 25 pediatric patients with MOGHE (m/f: 13/12) who underwent epilepsy surgery at a median age of 9.3 years at our center between 2003 and 2018. Median age at magnetic resonance imaging (MRI) was 5.2 years (1.5-20.7 years). RESULTS: Two MR subtypes were found: subtype I with an increased laminar T2 and fluid attenuated inversion recovery (FLAIR) signal at the corticomedullary junction and subtype II with reduced corticomedullary differentiation because of increased signal of the adjacent white matter. Distribution of subtypes was age-related, with subtype I occurring between 1.5 and 5.1 years (median 2.6 years) and subtype II between 3.4 and 20.7 years (median 14.1 years). In one patient, MRI at the age of 2.7 years showed subtype I but had converted to subtype II by the age of 16 years. Histology revealed that in addition to the above mentioned typical findings of MOGHE, patchy areas of reduced density of myelin in 6 of 7 patients presenting subtype I out of 14 patients in which retrospective analysis regarding myelination was accessible. CONCLUSION: Magnetic resonance characteristics in patients with MOGHE are age-related and seem to change from subtype I to subtype II probably because of maturational processes between 3 and 6 years. Patchy areas of hypomyelination in histology seem to disappear during brain maturation and may therefore represent the histologic correlate of laminar T2 and FLAIR hyperintensities in subtype I. This article is part of the Special Issue "Individualized Epilepsy Management: Medicines, Surgery and Beyond".
BACKGROUND: Mild malformation of cortical development with oligodendroglial hyperplasia and epilepsy (MOGHE) is a newly described, rare histopathologic entity detected in resected brain tissue of patients with refractory epilepsies. It shows a predominantly frontal localization causing a difficult-to-treat epilepsy with onset usually in early childhood. Histologically, MOGHE is characterized by blurred gray-white-matter boundaries with increased numbers of heterotopic neurons in the subcortical white matter and increased density of oligodendroglia. Little is known, to date, about radiologic features of MOGHE. Here, we report typical and age-related magnetic resonance (MR) characteristics of MOGHE. PATIENTS AND METHODS: Retrospective analysis of 40 preoperative MR images of 25 pediatric patients with MOGHE (m/f: 13/12) who underwent epilepsy surgery at a median age of 9.3 years at our center between 2003 and 2018. Median age at magnetic resonance imaging (MRI) was 5.2 years (1.5-20.7 years). RESULTS: Two MR subtypes were found: subtype I with an increased laminar T2 and fluid attenuated inversion recovery (FLAIR) signal at the corticomedullary junction and subtype II with reduced corticomedullary differentiation because of increased signal of the adjacent white matter. Distribution of subtypes was age-related, with subtype I occurring between 1.5 and 5.1 years (median 2.6 years) and subtype II between 3.4 and 20.7 years (median 14.1 years). In one patient, MRI at the age of 2.7 years showed subtype I but had converted to subtype II by the age of 16 years. Histology revealed that in addition to the above mentioned typical findings of MOGHE, patchy areas of reduced density of myelin in 6 of 7 patients presenting subtype I out of 14 patients in which retrospective analysis regarding myelination was accessible. CONCLUSION: Magnetic resonance characteristics in patients with MOGHE are age-related and seem to change from subtype I to subtype II probably because of maturational processes between 3 and 6 years. Patchy areas of hypomyelination in histology seem to disappear during brain maturation and may therefore represent the histologic correlate of laminar T2 and FLAIR hyperintensities in subtype I. This article is part of the Special Issue "Individualized Epilepsy Management: Medicines, Surgery and Beyond".
Authors: Irene Wang; Sehong Oh; Ingmar Blümcke; Roland Coras; Balu Krishnan; Sanghoon Kim; Aaron McBride; Olesya Grinenko; Yicong Lin; Margit Overmyer; Tin Tun Aung; Mark Lowe; Mykol Larvie; Andreas V Alexopoulos; William Bingaman; Jorge A Gonzalez-Martinez; Imad Najm; Stephen E Jones Journal: Epilepsia Date: 2020-09-19 Impact factor: 5.864
Authors: Imad Najm; Dennis Lal; Mario Alonso Vanegas; Fernando Cendes; Iscia Lopes-Cendes; Andre Palmini; Eliseu Paglioli; Harvey B Sarnat; Christopher A Walsh; Samuel Wiebe; Eleonora Aronica; Stéphanie Baulac; Roland Coras; Katja Kobow; J Helen Cross; Rita Garbelli; Hans Holthausen; Karl Rössler; Maria Thom; Assam El-Osta; Jeong Ho Lee; Hajime Miyata; Renzo Guerrini; Yue-Shan Piao; Dong Zhou; Ingmar Blümcke Journal: Epilepsia Date: 2022-06-15 Impact factor: 6.740