PDF(Pubmed)
Abstract：
Background: Focal-onset seizures and encephalopathy are prominent features of a stroke-like episode, which is a severe neurological manifestation associated with subtypes of mitochondrial disease. Despite more than 30 years of research, the acute treatment of stroke-like episodes remains controversial. Methods: We used the modified Delphi process to harness the clinical expertise of a group of mitochondrial disease specialists from five European countries to produce consensus guidance for the acute management of stroke-like episodes and commonly associated complications. Results: Consensus on a new definition of mitochondrial stroke-like episodes was achieved and enabled the group to develop diagnostic criteria based on clinical features, neuroimaging and/or electroencephalogram findings. Guidelines for the management of strokelike episodes were agreed with aggressive seizure management strongly recommended at the outset of stroke-like episodes. Conclusions: Our consensus statement defines stroke-like episodes in terms of an epileptic encephalopathy and we have used this to revise both diagnostic criteria and guidelines for management. A prospective, multi-centre, randomised controlled trial is required for evaluating the efficacy of any compound on modifying the trajectory of stroke-like episodes.
摘要：
背景：局灶性发作性癫痫和脑病是中风样发作的突出特征，这是与线粒体疾病亚型相关的严重神经系统表现。尽管进行了30多年的研究,卒中样发作的急性治疗仍存在争议.方法：我们使用改良的Delphi程序来利用来自五个欧洲国家的一组线粒体疾病专家的临床专业知识，为中风样发作和常见相关并发症的急性治疗提供共识指导。结果：就线粒体卒中样发作的新定义达成共识，并使该小组能够根据临床特征制定诊断标准。神经影像学和/或脑电图发现。中风样发作的管理指南与中风样发作开始时强烈建议的积极癫痫发作管理达成一致。结论：我们的共识声明根据癫痫性脑病定义了中风样发作，我们已将其用于修订诊断标准和治疗指南。一个潜在的，多中心,需要随机对照试验来评估任何化合物对改变卒中样发作轨迹的疗效.
