Fujita Tomoaki, Nishimura Shinjitsu, Sakata Hiroyuki, Furuno Yuichi, Mino Masaki, Hori Emiko, Kaimori Mitsuomi, Shirane Reizou, Nishijima Michiharu
Department of Neurosurgery, Aomori Prefectural Central Hospital, Japan.
No Shinkei Geka. 2009 Aug;37(8):781-5.
Gelastic seizure is a rare form of epilepsy defined as automatic bouts of laughter without mirth commonly associated with a hypothalamic hamartoma. Surgical treatment of hypothalamic hamartomas is associated with a high risk of complications because of the close vicinity of adjacent structures such as the optic tracts and mammillary bodies. This case was an 11-year-old girl who presented with gelastic seizure and complicated partial seizure. She developed gelastic seizure at a frequency of 10 bouts per day. She was found to have an elliptical mass close to the left hypothalamus. The signal intensity on magnetic resonance imaging (MRI) was consistent with hypothalamic hamartoma. The patient underwent surgical resection by a subtemporal approach. Pathological findings confirmed the diagnosis of hypothalamic hamartoma. Postoperative MRI demonstrated that the hypothalamic hamartoma was successfully resected. Twenty four months after surgery, complicated partial seizure in this patient has improved to Engel's class 1a and gelastic seizure has improved to Engel's class IIIa.
痴笑性癫痫是一种罕见的癫痫形式,定义为无愉快感的自动性发笑发作,通常与下丘脑错构瘤有关。由于视束和乳头体等相邻结构距离很近,下丘脑错构瘤的手术治疗并发症风险很高。该病例是一名11岁女孩,表现为痴笑性癫痫和复杂部分性发作。她每天发作痴笑性癫痫10次。发现她左下丘脑附近有一个椭圆形肿块。磁共振成像(MRI)上的信号强度与下丘脑错构瘤一致。患者通过颞下途径接受了手术切除。病理结果证实了下丘脑错构瘤的诊断。术后MRI显示下丘脑错构瘤已成功切除。术后24个月,该患者的复杂部分性发作改善至恩格尔1a级,痴笑性癫痫改善至恩格尔IIIa级。