Nishikura Noriko, Takano Tomoyuki, Ryujin Fukiko, Yoshioka Seiichiro, Maruo Yoshihiro, Takeuchi Yoshihiro, Tamura Kentaro, Sakakibara Takafumi, Ito Masayuki
Department of Pediatrics, Shiga University of Medical Science, Otsu, Japan.
Department of Developmental and Behavioral Pediatrics, Shiga University of Medical Science, Otsu, Japan.
Pediatr Int. 2016 Jun;58(6):496-500. doi: 10.1111/ped.12911. Epub 2016 May 10.
Dysembryoplastic neuroepithelial tumors (DNT) are benign hamartomatous tumors characterized by intractable epilepsy and common localization in the supratentorial cortex, but thalamic involvement in DNT is extremely rare. A 2-year 4-month-old boy presented with intractable epilepsy due to a tumorous lesion in the frontal lobe expanding to the thalamus. Under chronic intracranial electrocorticography guidance, partial lesionectomy with adjacent cortical resection was performed, and the lesion was pathologically diagnosed as DNT, complex form. Subsequently, the seizures completely disappeared without any neurological deficits despite the presence of full residual thalamic lesions. The epileptogenicity of DNT is closely associated with various clinicopathological factors, and the thalamic contribution to the seizure activity remains unclear. Due to the essential epileptogenic characteristics of DNT, the residual thalamic lesions and associated clinical features should be strictly observed in the future in the present case.
胚胎发育不良性神经上皮肿瘤(DNT)是一种良性错构瘤性肿瘤,其特征为顽固性癫痫且常见于幕上皮质,但丘脑受累的DNT极为罕见。一名2岁4个月大的男孩因额叶肿瘤性病变扩展至丘脑而出现顽固性癫痫。在慢性颅内皮层脑电图引导下,进行了部分病变切除术及相邻皮质切除术,病变经病理诊断为复杂型DNT。随后,尽管丘脑病变完全残留,但癫痫发作完全消失,且无任何神经功能缺损。DNT的致痫性与多种临床病理因素密切相关,丘脑对癫痫活动的作用仍不清楚。鉴于DNT的本质致痫特征,在本病例中,未来应严格观察残留的丘脑病变及相关临床特征。