神经皮肤黑变病合并部分性癫

Neurocutaneous melanosis with partial epilepsy:a case report and literature review

目的讨论神经皮肤黑变病(NCM)合并部分性癫的手术治疗及效果。方法回顾性分析1例NCM合并癫病人的临床资料。皮质电极监测后,行颞极及颞叶新皮质部分切除术。结果术后脑组织病理提示:颞极见大量黑色素细胞,原发性脑膜黑色素细胞增生症。皮肤病理提示复合性色素痣。术后10个月无癫发作。Engel分级Ⅰ级。结论 NCM是癫的罕见原因,但手术治疗效果满意。致灶切除术是一种安全有效的治疗方法。

Objective To discuss the surgical treatment and outcome of neurocutaneous melanosis associated with partial epilepsy.Methods Clinical data of 1 patient with neurocutaneous melanosis associated with partial epilepsy were analyzed retrospectively.The temporal pole monitoring and temporal neocortex partial resection was performed on patient after cortical electrode monitoring.Results The pathological results of the brain tissue revealed a lot of melanoma cells in the temporal pole and the disease was considered as primary meningeal melanocytosis.The pathological results of skin indicated compound-type pigmented nevus.The patient was followed up for 10 months,no seizure occurred and Engel grade was grade Ⅰ.Conclusions The neurocutaneous melanosis is a rare cause of epilepsy,but the surgical outcome is good.The resection of epileptogenic focus is a safe and effective method for neurocutaneous melanosis with partial epilepsy.