Lüders Hans, Schuele Stephan U
Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH 44195, USA.
Curr Opin Neurol. 2006 Apr;19(2):169-74. doi: 10.1097/01.wco.0000218234.67840.cb.
Patients with malformations of cortical development often suffer from intractable focal epilepsy. This review considers recent progress in the selection and seizure outcome of patients undergoing resective epilepsy surgery for this condition.
Patients with malformations of cortical development restricted to part or even a whole hemisphere may be candidates for epilepsy surgery even when, due to microscopic malformations, magnetic resonance imaging shows no detectable lesion. Despite recent advances in structural and functional imaging, the majority of patients with this condition undergo invasive evaluation. Patients with focal cortical dysplasia, with and without a detectable lesion on magnetic resonance imaging, often have a favorable outcome with epilepsy surgery. The underlying pathological substrate seems to be a better predictor for surgical outcome in patients with focal cortical dysplasia than the presence of a lesion on magnetic resonance imaging. Epilepsy surgery can be offered in a highly selected subgroup of patients with unilateral nodular heterotopia. Seizures in hemimegalencephaly may respond favorably to hemispherectomy, although most children will continue to have seizures and significant functional impairments.
Patients with focal epilepsy due to malformations of cortical development are often intractable to medical management. Resective epilepsy surgery can be beneficial, particularly for patients with focal cortical dysplasia and unilateral hemispheric malformations.
皮质发育畸形患者常患有难治性局灶性癫痫。本综述探讨了针对这种情况接受切除性癫痫手术患者的选择及癫痫发作结果的近期进展。
即使由于微小畸形，磁共振成像未显示可检测到的病变，局限于部分甚至整个半球的皮质发育畸形患者仍可能是癫痫手术的候选者。尽管结构和功能成像取得了最新进展，但大多数患有这种疾病的患者仍需进行侵入性评估。磁共振成像上有或无可检测到病变的局灶性皮质发育异常患者，癫痫手术通常有较好的结果。对于局灶性皮质发育异常患者，潜在的病理基础似乎比磁共振成像上病变的存在更能预测手术结果。在高度选择的单侧结节性异位患者亚组中可进行癫痫手术。半侧巨脑症患者的癫痫发作可能对半球切除术反应良好，尽管大多数儿童仍会继续发作并伴有明显的功能障碍。
因皮质发育畸形导致的局灶性癫痫患者通常药物治疗无效。切除性癫痫手术可能有益，特别是对局灶性皮质发育异常和单侧半球畸形患者。
