The clinicopathologic spectrum of focal cortical dysplasias: a consensus classification proposed by an ad hoc Task Force of the ILAE Diagnostic Methods Commission.

The clinicopathologic spectrum of focal cortical dysplasias: a consensus classification proposed by an ad hoc Task Force of the ILAE Diagnostic Methods Commission.
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DOI:
10.1111/j.1528-1167.2010.02777.x
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发表时间:
2011-01
期刊:
影响因子:
5.6
通讯作者:
Spreafico R
Spreafico R
中科院分区:
医学1区
文献类型:
--
作者:
Blümcke I;Thom M;Aronica E;Armstrong DD;Vinters HV;Palmini A;Jacques TS;Avanzini G;Barkovich AJ;Battaglia G;Becker A;Cepeda C;Cendes F;Colombo N;Crino P;Cross JH;Delalande O;Dubeau F;Duncan J;Guerrini R;Kahane P;Mathern G;Najm I;Ozkara C;Raybaud C;Represa A;Roper SN;Salamon N;Schulze-Bonhage A;Tassi L;Vezzani A;Spreafico R

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局灶性皮质发育不良(FCD)是畸形大脑皮质的局部区域,在儿童和成人中经常与癫痫相关。广泛的组织病理学已被纳入FCD的诊断。特征性表现包括新皮质的异常径向或切向分层(FCD I型)和细胞学异常(FCD II型)。ILAE工作组重新评估了现有数据,并提出了FCD的临床病理分类系统。自先前分类以来的主要变化是引入了FCD III型,其与海马硬化(FCD IIIa型)或癫痫相关肿瘤(FCD IIIb型)合并发生。IIIc型FCD与血管畸形相邻,而IIId型FCD可诊断为与早期获得的致癫痫病变相关(即,创伤性损伤、缺血性损伤或脑炎)。因此,FCD I型现在将指孤立的病变,其表现为新皮质的径向(FCD Ia型)或切向(FCD Ib型)分层障碍,显微镜下在一个或多个叶中识别。FCD II型是一种孤立性病变,其特征为皮质分层障碍和畸形神经元,无球囊细胞(IIa型)或有球囊细胞(IIb型)。邻近或在皮质发育的大体畸形内的结构异常经常被观察到,并且不被区分为特定的FCD变体。这三层分类系统将有助于更好地表征特定的临床病理实体,是进一步探索成像,电临床特征和术后癫痫控制以及潜在的分子病理机制的重要基础。
Focal cortical dysplasias (FCDs) are localized regions of malformed cerebral cortex and are very frequently associated with epilepsy in both children and adults. A broad spectrum of histopathology has been included in the diagnosis of FCD. Characteristic findings include aberrant radial or tangential lamination of the neocortex (FCD Type I) and cytological abnormalities (FCD Type II). An ILAE task force has re-evaluated available data and proposes a clinico-pathologic classification system of FCDs. The major change since a prior classification represents the introduction of FCD Type III, which occurs in combination with Hippocampal Sclerosis (FCD Type IIIa), or with epilepsy-associated tumors (FCD Type IIIb). FCD Type IIIc is found adjacent to vascular malformations, whereas FCD Type IIId can be diagnosed in association with epileptogenic lesions acquired in early life (i.e., traumatic injury, ischemic injury or encephalitis). Hence, FCD Type I will now refer to isolated lesions, which present either as radial (FCD Type Ia) or tangential (FCD Type Ib) dyslamination of the neocortex, microscopically identified in one or multiple lobes. FCD Type II is an isolated lesion characterized by cortical dyslamination and dysmorphic neurons without (Type IIa) or with balloon cells (Type IIb). Architectural abnormalities adjacent to or within gross malformations of cortical development are frequently observed and not distinguished as a specific FCD variant. This three-tiered classification system will help to better characterize specific clinico-pathological entities and is an important basis to further explore imaging, electro-clinical features, and postsurgical seizure control as well as underlying molecular pathomechanisms.
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