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Front Neurol. 2019 Jul 3;10:710. doi: 10.3389/fneur.2019.00710. eCollection 2019.

International Guidelines for the Treatment of Huntington's Disease.

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National Centre of Reference for Huntington's Disease, Henri Mondor Hospital, AP-HP, Creteil & NeurATRIS, Créteil, France.
Clinical Pharmacology Unit, Instituto de Medicina Molecular, Lisbon, Portugal.
IPMCN, School of Medicine, Cardiff University, Cardiff, United Kingdom.
Centre for Trials Research, Cardiff University, Cardiff, United Kingdom.
Division of Evolution and Genomic Sciences, Faculty of Biology, Medicine and Health, Manchester Centre for Genomic Medicine, School of Biological Sciences, University of Manchester, Manchester, United Kingdom.
Manchester Academic Health Science Centre, Saint Mary's Hospital, Manchester University NHS Foundation Trust, Manchester, United Kingdom.
Department of Radiology, George-Huntington-Institute, Universitaetsklinikum Muenster, Münster, Germany.
Department of Neurodegenerative Diseases and Hertie-Institute for Clinical Brain Research, University of Tuebingen, Tuebingen, Germany.
Department of Neurosciences, Federico II University, Naples, Italy.
Department of Clinical Genetics, NHS Grampian, Aberdeen, United Kingdom.
Huntington and Rare Diseases Unit, IRCCS Casa Sollievo della Sofferenza, San Giovanni Rotondo, Italy.
Department of Neurology, Medical University Innsbruck, Innsbruck, Austria.
Genetic Department, National Center of reference for Huntington's Disease, Salpêtrière Hospital, Paris, France.
Neurology Department, Angers University Hospital, Angers, France.
Neurology Department and UMR CNRS 6214 INSERM U1083, National Centre of Reference for Neurodegenerative Diseases, Angers University Hospital, Angers, France.
NeuroZentrumSiloah and Department of Neurology, Swiss HD Center, University of Bern, Bern, Switzerland.


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.


Huntington's disease; care; clinical practice; guidelines; treatment

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