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Huntington’s Disease 

Huntington’s Disease
Chapter:
Huntington’s Disease
Author(s):

Roger A. Barker

and Josef Priller

DOI:
10.1093/med/9780199609536.003.0020
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date: 03 December 2020

Huntington’s disease (HD) is a rare autosomal dominant neurodegenerative condition that commonly presents in mid life with a combination of a movement disorder and psychiatric and cognitive problems. The identification of the gene underlying HD in 1993 has led to many major breakthroughs in our understanding of this disease, but it remains incurable and fatal to the present time. Our better understanding of the range of clinical features seen in this complex neuropsychiatric disorder and our ability to detect those earliest changes is leading to a new era of drugs trials for HD with the prospect of identifying disease-modifying agents. There is also a new impetus to look at drugs to better treat the symptoms and signs of HD as well as better support patients and their families in the community. In this chapter we outline some of these therapies, and provide clinical tips on how best to assess the HD patient.

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