Kanai Sotaro, Okanishi Tohru, Nishimura Mitsuyo, Iijima Kentaro, Yokota Takuya, Yamazoe Tomohiro, Fujimoto Ayataka, Enoki Hideo, Yamamoto Takamichi
Department of Child Neurology, Seirei-Hamamatsu General Hospital, 2-12-12 Sumiyoshi, Hamamatsu, Shizuoka 430-8558, Japan.
Department of Child Neurology, Seirei-Hamamatsu General Hospital, 2-12-12 Sumiyoshi, Hamamatsu, Shizuoka 430-8558, Japan.
Brain Dev. 2017 Nov;39(10):882-885. doi: 10.1016/j.braindev.2017.06.001. Epub 2017 Jun 30.
Doose syndrome (epilepsy with myoclonic-atonic seizures) is an epilepsy syndrome with an incidence of approximately 1-2% of childhood-onset epilepsies. Although this syndrome is associated with multiple types of generalized seizures, the diagnosis is based on the presence of myoclonic-atonic seizures. Eighteen percent of patients have refractory seizures and intellectual disabilities. There have, however, been a few reports on the efficacy of surgical treatment for Doose syndrome. We describe a case of Doose syndrome in a 10-year-old boy. He developed generalized tonic-clonic seizures at 3years 8months of age and subsequently developed myoclonic-atonic, myoclonic, and tonic seizures. The frequent myoclonic seizures were refractory to multiple antiepileptic medications. His cognitive development was moderately delayed. Anterior four fifths corpus callosotomy was performed at 8years 5months of age. His seizures, especially myoclonic seizures, were markedly reduced. He was given vagus nerve stimulation therapy at 9years and 1month of age, which led to complete resolution of the myoclonic seizures. Corpus callosotomy can be a good treatment strategy in patients with Doose syndrome with medically refractory generalized seizures.
杜斯综合征（伴肌阵挛 - 失张力发作的癫痫）是一种癫痫综合征，在儿童期起病的癫痫中发病率约为1 - 2%。尽管该综合征与多种类型的全身性发作有关，但诊断基于肌阵挛 - 失张力发作的存在。18%的患者有难治性发作和智力障碍。然而，关于杜斯综合征手术治疗疗效的报道较少。我们描述了一名10岁男孩患杜斯综合征的病例。他在3岁8个月时出现全身性强直 - 阵挛发作，随后又出现肌阵挛 - 失张力发作、肌阵挛发作和强直发作。频繁的肌阵挛发作对多种抗癫痫药物治疗无效。他的认知发育中度延迟。在8岁5个月时进行了胼胝体前部五分之四切开术。他的发作，尤其是肌阵挛发作，明显减少。在9岁1个月时给他进行了迷走神经刺激治疗，这导致肌阵挛发作完全缓解。对于药物难治性全身性发作的杜斯综合征患者来说，胼胝体切开术可能是一种很好的治疗策略。
