Nass R, Gross A, Wisoff J, Devinsky O
Department of Neurology, New York University Medical Center, New York 10016, USA.
Pediatr Neurol. 1999 Jul;21(1):464-70. doi: 10.1016/s0887-8994(99)00029-6.
Treatment options for atypical forms of Landau-Kleffner syndrome (LKS) are not well delineated. Many patients with typical LKS fail to respond to antiepileptic drug treatment, but some benefit from multiple subpial transections (MSTs). The authors report seven patients with autism or autistic epileptiform regression who responded in varying degrees to MSTs after failed medical management. These patients derived from an original cohort of 36 children (29 males, seven females, ranging from 2 years, 3 months to 11 years, 3 months, mean age = 5 years, 8 months) with a history of language delay or regression, as well as varying degrees of social and behavioral abnormalities, who were evaluated with video-electroencephalogram (EEG) monitoring over a 2-year period. Fifteen patients had clinical seizures (11 of the 19 children with autistic epileptiform regression and four of 12 autistic children). Epilepsy was refractory to medication in seven. Surgical treatment variously involved MSTs of the left neocortex in temporal, parietal, and frontal regions, often including regions within the classic perisylvian language areas. One patient also had a left temporal lobectomy. In all seven patients, seizure control or EEG improved after MSTs. Language, social, and overall behavior improved to a moderate degree, although improvements were temporary in most. Autistic epileptiform regression resembles LKS in that both may respond to MST. MST is used to treat epilepsy in eloquent regions. The responsiveness of autistic epileptiform regression to MST buttresses the argument that autistic epileptiform regression is a form of focal epilepsy.
非典型性Landau-Kleffner综合征（LKS）的治疗方案尚未明确界定。许多典型LKS患者对抗癫痫药物治疗无反应，但有些患者从多处软膜下横切术（MST）中获益。作者报告了7例患有自闭症或自闭症性癫痫样退化的患者，在药物治疗失败后，他们对MST有不同程度的反应。这些患者来自最初的36名儿童队列（29名男性，7名女性，年龄从2岁3个月至11岁3个月，平均年龄=5岁8个月），有语言发育迟缓或退化史，以及不同程度的社交和行为异常，在2年期间通过视频脑电图（EEG）监测进行评估。15例患者有临床癫痫发作（19例自闭症性癫痫样退化儿童中的11例和12例自闭症儿童中的4例）。7例患者的癫痫对药物治疗无效。手术治疗包括在颞叶、顶叶和额叶区域对左侧新皮质进行不同程度的MST，通常包括经典的外侧裂周语言区域内的区域。1例患者还进行了左侧颞叶切除术。在所有7例患者中，MST后癫痫控制或EEG有所改善。语言、社交和整体行为有中度改善，尽管大多数改善是暂时的。自闭症性癫痫样退化与LKS相似，两者都可能对MST有反应。MST用于治疗明确区域的癫痫。自闭症性癫痫样退化对MST的反应支持了自闭症性癫痫样退化是局灶性癫痫一种形式的观点。
