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.
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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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