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Sturge-Weber Syndrome Is Associated with Cortical Dysplasia ILAE Type IIIc and Excessive Hypertrophic Pyramidal Neurons in Brain Resections for Intractable Epilepsy

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机构: [1]Department of Pathology, Xuanwu Hospital, Capital Medical University, [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,
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关键词: brain classification cortex malformation seizure

摘要:
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.

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出版当年[2014]版:
大类 | 2 区 医学
小类 | 2 区 临床神经病学 2 区 病理学 3 区 神经科学
最新[2023]版:
大类 | 2 区 医学
小类 | 1 区 病理学 2 区 临床神经病学 2 区 神经科学
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出版当年[2013]版:
Q1 PATHOLOGY Q1 CLINICAL NEUROLOGY Q1 NEUROSCIENCES
最新[2023]版:
Q1 CLINICAL NEUROLOGY Q1 NEUROSCIENCES Q1 PATHOLOGY

影响因子: 最新[2023版] 最新五年平均 出版当年[2013版] 出版当年五年平均 出版前一年[2012版] 出版后一年[2014版]

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第一作者机构: [1]Department of Pathology, Xuanwu Hospital, Capital Medical University,
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通讯机构: [*1]Department of Pathology, Xuanwu Hospital, Capital Medical University, No. 45 Changchun Street, Xicheng District, Beijing, 100053, China
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