Reinhard Carola, Bachoud-Lévi Anne-Catherine, Bäumer Tobias, Bertini Enrico, Brunelle Alicia, Buizer Annemieke I, Federico Antonio, Gasser Thomas, Groeschel Samuel, Hermanns Sanja, Klockgether Thomas, Krägeloh-Mann Ingeborg, Landwehrmeyer G Bernhard, Leber Isabelle, Macaya Alfons, Mariotti Caterina, Meissner Wassilios G, Molnar Maria Judit, Nonnekes Jorik, Ortigoza Escobar Juan Dario, Pérez Dueñas Belen, Renna Linton Lori, Schöls Ludger, Schuele Rebecca, Tijssen Marina A J, Vandenberghe Rik, Volkmer Anna, Wolf Nicole I, Graessner Holm
Institute for Medical Genetics and Applied Genomics, University of Tübingen, Tübingen, Germany.
Centre for Rare Diseases, University Hospital Tübingen, Tübingen, Germany.
Front Neurol. 2021 Jan 14;11:616569. doi: 10.3389/fneur.2020.616569. eCollection 2020.
While rare diseases (RDs) are by definition of low prevalence, the total number of patients suffering from an RD is high, and the majority of them have neurologic manifestations, involving central, peripheral nerve, and muscle. In 2017, 24 European Reference Networks (ERNs), each focusing on a specific group of rare or low-prevalence complex diseases, were formed to improve the care for patients with an RD. One major aim is to have "the knowledge travel instead of the patient," which has been put into practice by the implementation of the Clinical Patient Management System (CPMS) that enables clinicians to perform pan-European virtual consultations. The European Reference Network for Rare Neurological Diseases (ERN-RND) provides an infrastructure for knowledge sharing and care coordination for patients affected by a rare neurological disease (RND) involving the most common central nervous system pathological conditions. It covers the following disease groups: (i) Cerebellar Ataxias and Hereditary Spastic Paraplegias; (ii) Huntington's disease and Other Choreas; (iii) Frontotemporal dementia; (iv) Dystonia, (non-epileptic) paroxysmal disorders, and Neurodegeneration with Brain Iron Accumulation; (v) Leukoencephalopathies; and (vi) Atypical Parkinsonian Syndromes. At the moment, it unites 32 expert centers and 10 affiliated partners in 21 European countries, as well as patient representatives, but will soon cover nearly all countries of the European Union as a result of the ongoing expansion process. Disease expert groups developed and consented on diagnostic flowcharts and disease scales to assess the different aspects of RNDs. ERN-RND has started to discuss diagnostically unclear patients in the CPMS, is one of four ERNs that serve as foundation of Solve-RD, and has established an RND training and education program. The network will facilitate trial readiness through the establishment of an ERN-RND registry with a minimal data of all patients seen at the ERN-RND centers, thus providing a unique overview of existing genotype-based cohorts. The overall aim of the ERNs is to improve access for patients with RDs to quality diagnosis, care, and treatment. Based on this objective, ERNs are monitored by the European Commission on a regular basis to provide transparency and reassurance to the RD community and the general public.
虽然罕见病(RD)从定义上讲患病率较低,但患罕见病的患者总数却很多,且其中大多数有神经学表现,累及中枢、周围神经和肌肉。2017年,成立了24个欧洲参考网络(ERN),每个网络专注于一组特定的罕见或低患病率复杂疾病,以改善对罕见病患者的护理。一个主要目标是实现“让知识流动而非患者流动”,这已通过实施临床患者管理系统(CPMS)得以实践,该系统使临床医生能够进行泛欧洲虚拟会诊。欧洲罕见神经疾病参考网络(ERN-RND)为受罕见神经疾病(RND)影响的患者提供了知识共享和护理协调的基础设施,这些疾病涉及最常见的中枢神经系统病理状况。它涵盖以下疾病组:(i)小脑共济失调和遗传性痉挛性截瘫;(ii)亨廷顿舞蹈病和其他舞蹈病;(iii)额颞叶痴呆;(iv)肌张力障碍、(非癫痫性)发作性疾病和脑铁沉积神经变性;(v)白质脑病;以及(vi)非典型帕金森综合征。目前,它联合了21个欧洲国家的32个专家中心和10个附属合作伙伴以及患者代表,但由于正在进行的扩展进程,很快将覆盖几乎所有欧盟国家。疾病专家组制定并认可了诊断流程图和疾病量表,以评估罕见神经疾病的不同方面。ERN-RND已开始在CPMS中讨论诊断不明确的患者,是作为Solve-RD基础的四个ERN之一,并已建立了罕见神经疾病培训和教育计划。该网络将通过建立ERN-RND登记册促进试验准备工作,登记册包含在ERN-RND中心就诊的所有患者的最少数据,从而提供基于现有基因型队列的独特概述。欧洲参考网络的总体目标是改善罕见病患者获得高质量诊断、护理和治疗的机会。基于这一目标,欧盟委员会定期对欧洲参考网络进行监测,以向罕见病群体和公众提供透明度和保障。