Journal
ANNALS OF NEUROLOGY
Volume 82, Issue 3, Pages 479-483Publisher
WILEY
DOI: 10.1002/ana.25007
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Funding
- VENI grant from the Netherlands Organization of Scientific Research [91615080]
- Marie Sklodowska-Curie Individual Fellowship grant from the European Union (Horizon) [701130]
- CHDI Foundation
- Marie Curie Actions (MSCA) [701130] Funding Source: Marie Curie Actions (MSCA)
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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.
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