title: Fat-free mass and its predictors in Huntington's disease.
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.
authors.affiliation: Department of Neurology, Ulm University, Oberer Eselsberg 45/1, 89081, Ulm, Germany.
authors.forename: S D
authors.initials: SD
authors.lastname: Süssmuth
authors.forename: V M
authors.initials: VM
authors.lastname: Müller
authors.forename: C
authors.initials: C
authors.lastname: Geitner
authors.forename: G B
authors.initials: GB
authors.lastname: Landwehrmeyer
authors.forename: S
authors.initials: S
authors.lastname: Iff
authors.forename: A
authors.initials: A
authors.lastname: Gemperli
authors.forename: Michael
authors.initials: M
authors.lastname: Orth
mesh_terms: D000328:Adult; D001823:Body Composition; D015992:Body Mass Index; D002452:Cell Count; D019587:Dietary Supplements; D017097:Electric Impedance; D005223:Fats / Q000378:metabolism*; D005260:Female; D006801:Humans; D000071058:Huntingtin Protein; D006816:Huntington Disease / Q000175:diagnosis* / Q000235:genetics / Q000378:metabolism*; D008214:Lymphocytes / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009419:Nerve Tissue Proteins / Q000235:genetics; D015596:Nutrition Assessment; D011237:Predictive Value of Tests; D018911:Trinucleotide Repeats / Q000235:genetics
chemical_list: D005223:Fats; C086055:HTT protein, human; D000071058:Huntingtin Protein; D009419:Nerve Tissue Proteins