Tuesday 29 August 2017

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

These are very interesting results from another neurodegenerative disease, Huntington's (or HD). Although the authors use an observational study design, which like most observational studies can be susceptible to bias due to reverse causation and confounding, the results are very interesting. There is now evidence to suggest that higher BMI (note this is different to say obesity) appears to reduce risk of Parkinson's and Alzheimer's, and be associated with slower progression of HD and ALS. For PD at least in our paper the association was causal. 

Ann Neurol. 2017 Aug 5. doi: 10.1002/ana.25007. [Epub ahead of print]
van der Burg JMM, Gardiner SL, Ludolph AC, Landwehrmeyer GB, Roos RAC, Aziz NA.

http://onlinelibrary.wiley.com/doi/10.1002/ana.25007/full

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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