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Body weight is a robust predictor of clinical progression in Huntington disease

Van der Burg, JMM; Gardiner, SL; Ludolph, AC; Landwehrmeyer, GB; Roos, RA; Aziz, NA; (2017) Body weight is a robust predictor of clinical progression in Huntington disease. Annals of Neurology , 82 (3) pp. 479-483. 10.1002/ana.25007. Green open access

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Abstract

Unintended weight loss is a hallmark of Huntington disease (HD), but it is unknown to what extent weight loss impacts the rate of disease progression. Therefore, using longitudinal data from the Enroll-HD study, we assessed the association between baseline body mass index (BMI) and the rate of clinical progression in 5,821 HD mutation carriers. We found that high baseline BMI was associated with a significantly slower rate of functional, motor, and cognitive deterioration (all p < 0.001), independent of mutant HTT CAG repeat size. Our findings provide strong rationale for exploration of systemic metabolism as a therapeutic target in HD.

Type: Article
Title: Body weight is a robust predictor of clinical progression in Huntington disease
Open access status: An open access version is available from UCL Discovery
DOI: 10.1002/ana.25007
Publisher version: http://dx.doi.org/10.1002/ana.25007
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: Huntington disease, disease progression, weight loss, body mass index, metabolism, bioenergetics, polyglutamine disorders
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
URI: https://discovery-pp.ucl.ac.uk/id/eprint/1570815
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