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Focal cortical dysplasia type 1
Brain Pathology ( IF 6.4 ) Pub Date : 2021-07-01 , DOI: 10.1111/bpa.12964
Roland Coras 1 , Hans Holthausen 2 , Harvey B Sarnat 3
Affiliation  

The ILAE classification of Focal Cortical Dysplasia (FCD) from 2011 has quickly gained acceptance in clinical practice and research and is now widely used around the world. This histopathology-based classification scheme proposed three subtypes, that is, FCD Type 1 (with architectural abnormalities of the neocortex), FCD Type 2 (with cytoarchitectural abnormalities of the neocortex) and FCD Type 3 (architectural abnormalities of the neocortex associated with another principle lesion acquired during early life). Valuable knowledge was gathered during the last decade validating the clinical, pathological and genetic classification of FCD Type 2. This is in contrast to FCD subtype 1 and 3 with only few robust or new insights. Herein, we provide an overview about current knowledge about FCD Type 1 and its three subtypes. Available data strengthened, however, FCD Type 1A in particular, whereas a comprehensive clinico-pathological specification for FCD Type 1B and 1C subtypes remain to be shown. The lack of a valid animal model for FCD Type 1 further supports our call and the ongoing need for systematic research studies based on a careful clinico-pathological and genetic stratification of patients and human brain tissues.

中文翻译:

1型局灶性皮质发育不良

从 2011 年开始,ILAE 对局灶性皮质发育不良 (FCD) 的分类迅速在临床实践和研究中获得认可,现已在世界范围内广泛使用。这种基于组织病理学的分类方案提出了三种亚型,即FCD 1型(新皮质结构异常)、FCD 2型(新皮质细胞结构异常)和FCD 3型(新皮质结构异常与另一个原理相关)早年获得的损伤)。在过去十年中收集了宝贵的知识,验证了 FCD 2 型的临床、病理学和遗传分类。这与 FCD 亚型 1 和 3 形成对比,只有很少的可靠或新见解。在这里,我们概述了有关 FCD 类型 1 及其三个亚型的当前知识。现有数据得到加强,然而,特别是 FCD 1A 型,而 FCD 1B 型和 1C 型亚型的综合临床病理学规范仍有待显示。缺乏有效的 FCD 1 型动物模型进一步支持了我们的呼吁以及对基于患者和人类脑组织的仔细临床病理学和遗传分层的系统研究的持续需求。
更新日期:2021-07-01
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