Blümcke I, Mühlebner A
Department of Neuropathology, University Hospital Erlangen, Germany.
Clin Neuropathol. 2011 Jul-Aug;30(4):164-77. doi: 10.5414/np300398.
FCDs are increasingly recognized in patients with drug-resistant epilepsies, and many patients benefit from tailored resection strategies. Yet, postsurgical seizure control cannot be sufficiently predicted and specification of FCD variants remains difficult during presurgical monitoring. The International League against Epilepsy (ILAE) has published a new consensus classification system for focal cortical dysplasias (FCDs). Based on a review of imaging data, electroclinical features and postsurgical seizure control correlation with neuropathological findings specify three clinico-pathological FCD subtypes: FCD Type I is characterized by aberrant radial (FCD Type Ia) or tangential lamination of the neocortex (FCD Type Ib) affecting one or multiple lobes. FCD Type II is characterized by cortical dyslamination and dysmorphic neurons without (Type IIa) or with balloon cells (Type IIb). It is important to note, that these types should not be associated with any other structural brain lesion (isolated FCD). In contrast, a new FCD Type III is introduced, 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 other epileptogenic lesions obtained in early life (i.e., traumatic injury, ischemic injury or encephalitis). Histopathological features are very similar to those observed in FCD Type I, but likely present postnatal development and maturation failures acquired by the principal lesion. This first international consensus classification may encourage neuropathologists to focus their attention onto this important histopathological group. Addressing more precisely defined clinico-pathological entities will also help to clarify underlying pathomechanisms and, thereby, improve treatment strategies for patients with difficult-to-treat epilepsies.
局灶性皮质发育不良（FCD）在耐药性癫痫患者中越来越受到认可，许多患者受益于量身定制的切除策略。然而，术后癫痫发作控制仍无法得到充分预测，并且在术前监测期间确定FCD变体仍然很困难。国际抗癫痫联盟（ILAE）发布了一种新的局灶性皮质发育不良（FCD）共识分类系统。基于对影像学数据、电临床特征以及术后癫痫发作控制与神经病理学结果相关性的回顾，确定了三种临床病理FCD亚型：FCD I型的特征是新皮质的放射状异常（FCD Ia型）或切线分层异常（FCD Ib型），累及一个或多个脑叶。FCD II型的特征是皮质分层紊乱和异形神经元，无气球样细胞（IIa型）或有气球样细胞（IIb型）。需要注意的是，这些类型不应与任何其他脑结构性病变相关联（孤立性FCD）。相比之下，引入了一种新的FCD III型，它与海马硬化（FCD IIIa型）或与癫痫相关肿瘤（FCD IIIb型）同时出现。FCD IIIc型在血管畸形附近发现，而FCD IIId型可与早年获得的其他致痫性病变（即创伤性损伤、缺血性损伤或脑炎）相关联进行诊断。组织病理学特征与在FCD I型中观察到的非常相似，但可能呈现出由主要病变导致的出生后发育和成熟失败。这第一个国际共识分类可能会鼓励神经病理学家将注意力集中在这个重要的组织病理学组上。更精确地定义临床病理实体也将有助于阐明潜在的发病机制，从而改善难治性癫痫患者的治疗策略。
