3 years ago

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

Sarah L. Gardiner, Albert C. Ludolph, G. Bernhard Landwehrmeyer, N. Ahmad Aziz, Raymund A. C. Roos, Jorien M. M. van der Burg
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. Ann Neurol 2017;82:479–483

Publisher URL: http://onlinelibrary.wiley.com/resolve/doi

DOI: 10.1002/ana.25007

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