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

Ann Neurol. 2017 Sep;82(3):479-483. doi: 10.1002/ana.25007. Epub 2017 Aug 22.


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.

Publication types

  • Observational Study

MeSH terms

  • Body Mass Index*
  • Body Weight / physiology*
  • Cohort Studies
  • Disease Progression
  • Female
  • Humans
  • Huntington Disease / pathology
  • Huntington Disease / physiopathology*
  • Huntington Disease / psychology
  • Male
  • Middle Aged
  • Neuropsychological Tests
  • Weight Loss / physiology