Nariai Hiroki, Duberstein Susan, Shinnar Shlomo
1 Saul R. Korey Department of Neurology, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, New York, USA.
2 Department of Pediatrics, Montefiore Medical Center, Albert Einstein College of Medicine, Bronx, New York, NY, USA.
J Child Neurol. 2018 Jan;33(1):41-54. doi: 10.1177/0883073817690290. Epub 2017 Jan 30.
Childhood epileptic encephalopathies are age-dependent disorders of the brain whose hallmarks include loss of neurologic function over time, abnormal electroencephalographic findings, and seizures. Ictal and interictal electrographic activity are conjointly thought to be at the root of the often devastating neuropsychological deterioration, which is specific to the maturing brain. The goals of treatment are not only to control seizures, but also to prevent or reverse neurologic loss of function. In general, time is of the essence in diagnosis, and experienced specialists should promptly design a treatment plan. Hormonal and immune therapies are at the forefront of treatment in many cases, with traditional antiepileptic drugs and surgery (when an identifiable lesion is present) playing a limited role. However, gold standard evidence for treatment of epileptic encephalopathies remains limited. Ongoing clinical and basic research may lead to better understanding of these catastrophic conditions and to better and more effective therapies.
儿童癫痫性脑病是一种与年龄相关的脑部疾病,其特征包括随着时间推移神经功能丧失、脑电图检查结果异常以及癫痫发作。发作期和发作间期的脑电图活动被共同认为是通常具有毁灭性的神经心理衰退的根源,这种衰退是成熟大脑所特有的。治疗目标不仅是控制癫痫发作,还包括预防或逆转神经功能丧失。一般来说,诊断刻不容缓,经验丰富的专家应迅速制定治疗方案。在许多情况下,激素和免疫疗法处于治疗前沿,传统抗癫痫药物和手术(当存在可识别病变时)发挥的作用有限。然而,癫痫性脑病治疗的金标准证据仍然有限。正在进行的临床和基础研究可能会增进对这些灾难性疾病的了解,并带来更好、更有效的治疗方法。