Evidence base: Huntington's disease

A summary of the clinical trials, guidelines, key texts and online resources to help in the management of Huntington's disease

Scans of Huntington's disease showing neuronal loss (green areas)

Current research

Studies are focused on:

    * Developing a better understanding of the pathophysiology of the disease and identifying pathways which could be possible targets for disease-modifying intervention.9

    * Developing sensitive biomarkers to monitor the disease's course and measure the effects of modifying treatments. The two major international studies are Track-HD10 and Predict-HD.11

    * Identifying treatments to alter the disease's course. Previous trials of compounds have not demonstrated efficacy.12 A multicentre phase III trial of latrepirdine concluded in April 2011 but no significant improvements were identified (www.euro-hd.net/html/projects/horizon).

    * However, promising interventions are in the pipeline, and a European phase 1b trial of a compound known as SEN0014196 is currently recruiting (www.paddingtonproject.eu).



    * An evidence-based care map is available on the Map of Medicine website: http://eng.mapofmedicine.com/evidence/map/ huntington_s_disease1.html

    * A Cochrane review of medical treatment: Mestre T, Ferreira J, Coelho MM et al. Therapeutic interventions for symptomatic treatment in Huntington's disease. Cochrane Database Syst Rev 2009; 3: CD006456


Key text

    * Bates G, Harper P, Jones L (eds). Huntington's Disease (third edition). Oxford, Oxford University Press, 2002.


Online resources

    * A Physician's Guide to the Management of Huntington's Disease can be downloaded, free of charge, via the Huntington's Disease Association website: www.hda.org.uk/download/professionals/ HD-Management-Physicians-Guide.doc

    * Huntington's disease research news: www.HDBuzz.net


Contributed by Rachel Taylor, nurse consultant, The National Hospital for Neurology and Neurosurgery, and Professor Sarah Tabrizi, professor of neurology, UCL Institute of Neurology.

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