Premium
Body weight is a robust predictor of clinical progression in Huntington disease
Author(s) -
van der Burg Jorien M. M.,
Gardiner Sarah L.,
Ludolph Albert C.,
Landwehrmeyer G. Bernhard,
Roos Raymund A. C.,
Aziz N. Ahmad
Publication year - 2017
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.25007
Subject(s) - huntington's disease , body mass index , weight loss , medicine , disease , oncology , body weight , weight gain , psychology , obesity
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