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Neurodegenerative disease

Mapping the natural history of Huntington disease

Nature Reviews Neurology volume 10, pages 12–13 (2014)Cite this article

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A key challenge to improving the design of clinical trials in Huntington disease (HD) has been the limited data on the natural history of HD. A recent prospective longitudinal study has provided important information in this regard, which could be useful for future translation of disease-modifying therapies for early-stage HD.

Huntington disease (HD) is an autosomal dominant condition that typically presents in middle age with a combination of movement, cognitive and psychiatric problems, and leads to death 20–25 years later. HD is currently incurable, and although the underlying gene defect was discovered 20 years ago, its pathogenesis remains the subject of debate. Considerable efforts have been made to develop disease-modifying treatments to slow down disease progression and even delay onset, with many promising therapies emerging from the laboratory.1 Translation of such therapies to the clinic, however, is not straightforward, largely because easily accessible measures by which to track progression of this CNS disease are not available.

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  1. Department of Clinical Neurosciences, John van Geest Centre for Brain Repair, Forvie Site, Robinson Way, University of Cambridge, Cambridge, CB2 0PY, UK

    Roger A. Barker & Sarah L. Mason

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  1. Roger A. Barker
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  2. Sarah L. Mason
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Correspondence to Roger A. Barker.

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The authors declare no competing financial interests.

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Barker, R., Mason, S. Mapping the natural history of Huntington disease. Nat Rev Neurol 10, 12–13 (2014). https://doi.org/10.1038/nrneurol.2013.253

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