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Electro-clinical and imaging characteristics of focal cortical dysplasia: correlation with pathological subtypes.

机译:局灶性皮质发育异常的电临床和影像学特征:与病理亚型的相关性。

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INTRODUCTION: 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. METHODS: 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). RESULTS: 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. CONCLUSIONS: Clinically important differences exist between the pathological subtypes of CD, which may assist in their management, and provide further insight into their underlying pathophysiology.
机译:简介:局灶性皮质发育不良(CD)是药物耐药性癫痫的常见原因。 CD是由于神经元迁移,增殖和/或分化异常所致,导致四种不同的病理亚型:1A,1B,2A和2B。为了提供与这些病理亚型的临床相关性,我们审查了CD的四种病理亚型的电临床和影像学特征以及手术结局。方法:我们回顾性分析了1990年至2002年期间在克利夫兰诊所基金会的癫痫手术中获得的患者数据。该研究仅包括那些明确病理诊断为孤立性皮质发育异常的患者(n = 145)。结果:病理亚型2A和2B主要位于额叶,比1A和1B亚型具有更严重的癫痫综合征和较低的智商得分。患有1A FCD亚型的患者病情较轻,较晚发作,主要位于颞叶。癫痫的危险因素包括1A型高热惊厥,1A和1B型头部外伤以及2B型围产期不良事件。 2B型表现出比其他组明显更多的FLAIR信号异常。六个月的随访中,总体有63%的患者有Engel I结局。最好的结果是在2B亚型中,以及不需要进行侵入性脑电图评估的患者。结论:CD的病理亚型之间存在重要的临床差异,这可能有助于CD的管理,并进一步了解其潜在的病理生理学。

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