Sturge-Weber Syndrome Is Associated with Cortical Dysplasia ILAE Type IIIc and Excessive Hypertrophic Pyramidal Neurons in Brain Resections for Intractable Epilepsy
机构:[1]Department of Pathology, Xuanwu Hospital, Capital Medical University,病理科首都医科大学宣武医院[2]Department of Neuropathology, University Hospital Erlangen, Erlangen, Germany.[3]Department of Neurosurgery,Tsinghua University Yuquan Hospital,[4]Department of Pathology, PLA General Hospital, Beijing, China[5]Department of Pathology, Tsinghua University Yuquan Hospital,
Sturge-Weber syndrome (SWS) is a rare syndrome characterized by capillary-venous malformations involving skin and brain. Many patients with SWS also suffer from drug-resistant epilepsy. We retrospectively studied a series of six SWS patients with epilepsy and extensive neurosurgical resections. At time of surgery, the patients' age ranged from 11 to 35 years (with a mean of 20.2 years). All surgical specimens were well preserved, which allowed a systematic microscopical inspection utilizing the 2011 ILAE classification for focal cortical dysplasia (FCD). Neuropathology revealed dysmorphic-like neurons with hypertrophic cell bodies reminiscent to those described for FCD type IIa in all cases. However, gross architectural abnormalities of neocortical layering typical for FCD type IIa were missing, and we propose to classify this pattern as FCD ILAE type IIIc. In addition, our patients with earliest seizure onset also showed polymicrogyria (PMG; n=4). The ictal onset zones were identified in all patients by subdural electrodes, and these areas always showed histopathological evidence for FCD type IIIc. Four out of five patients had favorable seizure control after surgery with a mean follow-up period of 1.7 years. We concluded from our study that FCD type IIIc and PMG are frequently associated findings in SWS. FCD type IIIc may play a major epileptogenic role in SWS and complete resection of the associated FCD should be considered a prognostic key factor to achieve seizure control.
基金:
the Beijing Municipal Health Bureau“215 project” (No. 2011-3-095),
the German Research Foundation(DFG Bl421/3-1; EpiGenNet)
European Union FP7 Health program (agreement contract number 602531; DESIRE).
第一作者机构:[1]Department of Pathology, Xuanwu Hospital, Capital Medical University,
通讯作者:
通讯机构:[*1]Department of Pathology, Xuanwu Hospital, Capital Medical University, No. 45 Changchun Street, Xicheng District, Beijing, 100053, China
推荐引用方式(GB/T 7714):
Dan-Dan Wang,Ingmar Blümcke,Roland Coras,et al.Sturge-Weber Syndrome Is Associated with Cortical Dysplasia ILAE Type IIIc and Excessive Hypertrophic Pyramidal Neurons in Brain Resections for Intractable Epilepsy[J].BRAIN PATHOLOGY.2015,25(3):248-255.doi:10.1111/bpa.12172.
APA:
Dan-Dan Wang,Ingmar Blümcke,Roland Coras,Wen-Jing Zhou,De-Hong Lu...&Yue-Shan Piao.(2015).Sturge-Weber Syndrome Is Associated with Cortical Dysplasia ILAE Type IIIc and Excessive Hypertrophic Pyramidal Neurons in Brain Resections for Intractable Epilepsy.BRAIN PATHOLOGY,25,(3)
MLA:
Dan-Dan Wang,et al."Sturge-Weber Syndrome Is Associated with Cortical Dysplasia ILAE Type IIIc and Excessive Hypertrophic Pyramidal Neurons in Brain Resections for Intractable Epilepsy".BRAIN PATHOLOGY 25..3(2015):248-255