Table_1_International Guidelines for the Treatment of Huntington's Disease.XLSX

The European Huntington's Disease Network (EHDN) commissioned an international task force to provide global evidence-based recommendations for everyday clinical practice for treatment of Huntington's disease (HD). The objectives of such guidelines are to standardize pharmacological, surgical and non-pharmacological treatment regimen and improve care and quality of life of patients. A formalized consensus method, adapted from the French Health Authority recommendations was used. First, national committees (French and English Experts) reviewed all studies published between 1965 and 2015 included dealing with HD symptoms classified in motor, cognitive, psychiatric, and somatic categories. Quality grades were attributed to these studies based on levels of scientific evidence. Provisional recommendations were formulated based on the strength and the accumulation of scientific evidence available. When evidence was not available, recommendations were framed based on professional agreement. A European Steering committee supervised the writing of the final recommendations through a consensus process involving two rounds of online questionnaire completion with international multidisciplinary HD health professionals. Patients' associations were invited to review the guidelines including the HD symptoms. Two hundred and nineteen statements were retained in the final guidelines. We suggest to use this adapted method associating evidence base–medicine and expert consensus to other rare diseases.

欧洲亨廷顿病网络（EHDN）委托国际专家小组，旨在为亨廷顿病（HD）的日常临床实践提供全球循证推荐。此类指南的目标在于标准化药物、手术及非药物治疗方案，以提升患者护理质量与生活质量。本指南采用了一种正式化的共识方法，该方法源自法国卫生当局的推荐。首先，国家委员会（法国及英国专家）审阅了1965年至2015年间发表的所有关于亨廷顿病症状的研究，这些研究被分为运动、认知、精神病学和躯体类别。根据科学证据的水平，对这些研究赋予了质量等级。基于现有科学证据的强度与累积，形成了临时推荐。当缺乏证据时，基于专业共识制定推荐。欧洲指导委员会通过两轮在线问卷调查，监督了最终推荐的撰写，其中涉及国际多学科亨廷顿病卫生专业人士的共识过程。患者协会被邀请审阅包括亨廷顿病症状在内的指南。最终指南中保留了219项声明。我们建议将此结合循证医学与专家共识的方法应用于其他罕见疾病。