Mild Malformation of Cortical Development with Oligodendroglial Hyperplasia in Frontal Lobe Epilepsy: A New Clinico-Pathological Entity

详细信息    查看全文

• 作者：Johannes Schurr ; Roland Coras ; Karl Rö ; ssler ; Tom Pieper ; Manfred Kudernatsch ; Hans Holthausen ; Peter Winkler ; Friedrich Woermann ; Christian G. Bien ; Tilman Polster ; Reinhard Schulz ; Thilo Kalbhenn ; Horst Urbach ; Albert Becker ; Thomas Grunwald ; Hans-Juergen Huppertz ; Antonio Gil-Nagel ; Rafael Toledano ; Martha Feucht ; Angelika Mü ; hlebner ; Thomas Czech and Ingmar Blü ; mcke
• 刊名：Brain Pathology
• 出版年：2017
• 出版时间：January 2017
• 年：2017
• 卷：27
• 期：1
• 页码：26-35
• 全文大小：746K
• ISSN：1750-3639

文摘

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.