Mild Malformation of Cortical Development with Oligodendroglial Hyperplasia in Frontal Lobe Epilepsy: A New Clinico-Pathological Entity
| Autor: | Horst Urbach, Thilo Kalbhenn, Thomas Grunwald, Christian G. Bien, Roland Coras, Tom Pieper, Tilman Polster, Martha Feucht, Angelika Mühlebner, Peter A. Winkler, Johannes Schurr, Reinhard Schulz, Albert J. Becker, Antonio Gil-Nagel, Karl Rössler, Thomas Czech, Hans Holthausen, Rafael Toledano, Ingmar Blümcke, Hans-Juergen Huppertz, Friedrich G. Woermann, Manfred Kudernatsch |
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| Rok vydání: | 2016 |
| Předmět: |
0301 basic medicine
medicine.medical_specialty Pathology medicine.diagnostic_test business.industry General Neuroscience Magnetic resonance imaging Neuropathology Cortical dysplasia medicine.disease Pathology and Forensic Medicine White matter 03 medical and health sciences Epilepsy 030104 developmental biology 0302 clinical medicine medicine.anatomical_structure Frontal lobe medicine Epilepsy surgery Histopathology Neurology (clinical) business Neuroscience 030217 neurology & neurosurgery |
| Zdroj: | Brain Pathology. 27:26-35 |
| ISSN: | 1015-6305 |
| DOI: | 10.1111/bpa.12347 |
| Popis: | The histopathological spectrum of human epileptogenic brain lesions is widespread including common and rare variants of cortical malformations. However, 2-26% of epilepsy surgery specimens are histopathologically classified as nonlesional. We hypothesized that these specimens include also new diagnostic entities, in particular when presurgical magnetic resonance imaging (MRI) can identify abnormal signal intensities within the anatomical region of seizure onset. In our series of 1381 en bloc resected epilepsy surgery brain specimens, 52 cases could not be histopathologically classified and were considered nonlesional (3.7%). An increase of Olig2-, and PDGFR-alpha-immunoreactive oligodendroglia was observed in white matter and deep cortical layers in 22 of these patients (42%). Increased proliferation activity as well as heterotopic neurons in white matter were additional histopathological hallmarks. All patients suffered from frontal lobe epilepsy (FLE) with a median age of epilepsy onset at 4 years and 16 years at epilepsy surgery. Presurgical MRI suggested focal cortical dysplasia (FCD) in all patients. We suggest to classify this characteristic histopathology pattern as "mild malformation of cortical development with oligodendroglial hyperplasia (MOGHE)." Further insights into pathomechanisms of MOGHE may help to bridge the diagnostic gap in children and young adults with difficult-to-treat FLE. |
| Databáze: | OpenAIRE |
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