Abstract
The causes of weight loss in Huntington’s disease (HD) are not entirely clear. The aim was to identify risk factors that are associated with a loss of metabolically active tissues, i.e. fat-free mass. A consecutive cohort of non-diabetic HD participants (manifest HD, n = 43; CAG: mean 43.6.0 ± 3.6; preHD, n = 10; CAG: mean 41.4 ± 1.4) and 36 healthy controls was recruited. Twenty-five HD participants were early-stage HD (UHDRS Total Functional Capacity [TFC] stages I and II), 12 mid-stage HD (TFC stage III), and 6 participants were in late-stage HD (TFC stages IV and V). Food intake, basic metabolic rate and glucose homeostasis were assessed. In addition, fat-free mass was determined using bioelectric impedance analysis, and leptin, insulin and ghrelin as key metabolic regulators. Sex ratio and age were similar in HD participants (71 % women; age 50.6 ± 10.9) and controls (66 % women; age 46.4 ± 14.5). Body mass index (BMI) was lower in HD participants than controls (median 24.1 vs. 25.9, p = 0.04). However, fat-free mass and basic metabolic rate were not statistically different between groups and showed no association with disease burden. In controls and HD participants, leptin was the most important predictor of fat-free mass. While BMI was lower in HD participants, fat-free mass was similar to controls with leptin as its most important predictor. Leptin levels and fat-free mass measurements using bioelectric impedance analysis may be good screening tools to identify HD patients at risk for weight loss.
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Acknowledgments
We thank all participants for their time and effort in supporting this study.
Conflicts of interest
GB Landwehrmeyer declares consultancies from: Bayer Pharma AG, CHDI, GlaxoSmithKline, Hoffmann-LaRoche, Ipsen, Neurosearch Inc, Medesis, Medtronic, Novartis, Pfizer, Prana Biotechnology, Sangamo/Shire, Siena Biotech and TEVA. Advisory Boards: Ipsen, Neurosearch Inc, Medivation, Pfizer, Siena Biotech and TEVA. Honoraria: AOP Orphan Pharmaceuticals AG, Temmler Pharma GmbH. Grants: CHDI Foundation, the Bundesministerium für Bildung und Forschung (BMBF), the Deutsche Forschungsgemeinschaft (DFG), the European Commission (EU-FP7). M Orth is in receipt of grants from CHDI Foundation and the European Commission (EU-FP7). The other authors report no conflict of interest.
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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. This article does not contain any studies with animals performed by any of the authors.
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Informed consent was obtained from all participants included in the study.
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Süssmuth, S.D., Müller, V.M., Geitner, C. et al. Fat-free mass and its predictors in Huntington’s disease. J Neurol 262, 1533–1540 (2015). https://doi.org/10.1007/s00415-015-7753-0
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DOI: https://doi.org/10.1007/s00415-015-7753-0