Widdess-Walsh Peter, Kellinghaus Christoph, Jeha Lara, Kotagal Prakash, Prayson Richard, Bingaman William, Najm Imad M
Section of Epilepsy, Department of Neurology, The Cleveland Clinic Foundation, 9500 Euclid Avenue, S51, Cleveland, OH 44195, USA.
Epilepsy Res. 2005 Oct-Nov;67(1-2):25-33. doi: 10.1016/j.eplepsyres.2005.07.013. Epub 2005 Sep 21.
Focal cortical dysplasia (CD) is a common cause of pharmaco-resistant epilepsy. CD is due to abnormalities in neuronal migration, proliferation, and/or differentiation that result in four distinct pathological subtypes: 1A, 1B, 2A, and 2B. In order to provide clinical correlation to these pathological subtypes, we reviewed the electro-clinical and imaging characteristics and surgical outcomes of the four pathological subtypes of CD.
We retrospectively reviewed patient data from epilepsy surgeries at the Cleveland Clinic Foundation between 1990 and 2002. Only those patients with the definite pathological diagnosis of isolated cortical dysplasia were included in the study (n = 145).
Pathological subtypes 2A and 2B were predominantly frontal in location, and had a more severe epilepsy syndrome with lower intelligence quotient scores than subtypes 1A and 1B. Patients with subtype 1A FCD had less severe, later onset epilepsy that was predominantly located in the temporal lobe. Risk factors for epilepsy included febrile seizures for type 1A, head trauma for types 1A and 1B, and perinatal adverse events for type 2B. Type 2B demonstrated significantly more FLAIR signal abnormalities than the other groups. Sixty-three percent of patients overall had an Engel I outcome at 6 months follow-up. The best outcomes were in the 2B subtype, and in those who did not require an invasive EEG evaluation.
Clinically important differences exist between the pathological subtypes of CD, which may assist in their management, and provide further insight into their underlying pathophysiology.
局灶性皮质发育不良（CD）是药物难治性癫痫的常见病因。CD是由神经元迁移、增殖和/或分化异常引起的，导致四种不同的病理亚型：1A、1B、2A和2B。为了将这些病理亚型与临床相关联，我们回顾了CD四种病理亚型的电临床、影像学特征及手术结果。
我们回顾性分析了1990年至2002年克利夫兰诊所基金会癫痫手术患者的数据。本研究仅纳入那些明确病理诊断为孤立性皮质发育不良的患者（n = 145）。
病理亚型2A和2B主要位于额叶，与亚型1A和1B相比，具有更严重的癫痫综合征，智商得分更低。1A亚型FCD患者的癫痫症状较轻，发病较晚，主要位于颞叶。癫痫的危险因素包括1A亚型的热性惊厥、1A和1B亚型的头部外伤以及2B亚型的围产期不良事件。2B亚型的FLAIR信号异常明显多于其他组。总体上，63%的患者在6个月随访时达到恩格尔I级结果。最佳结果出现在2B亚型以及那些不需要进行侵入性脑电图评估的患者中。
CD的病理亚型之间存在临床上重要的差异，这可能有助于其管理，并为其潜在的病理生理学提供进一步的见解。
