title: Neuroendocrine disturbances in Huntington's disease.
abstract: BACKGROUND
  Huntington's disease (HD) is a severe inherited neurodegenerative disorder characterized, in addition to neurological impairment, by weight loss suggesting endocrine disturbances. The aims of this study were to look for neuroendocrine disturbances in patients with Huntington's disease (HD) and to determine the relationship with weight loss seen in HD METHODS AND FINDING: We compared plasma levels of hormones from the five pituitary axes in 219 patients with genetically documented HD and in 71 sex- and age-matched controls. Relationships between hormone levels and disease severity, including weight-loss severity, were evaluated. Growth hormone (GH) and standard deviation score of insulin-like growth factor 1 (SDS IGF-1) were significantly higher in patients than in controls (0.25 (0.01-5.89) vs. 0.15 (0.005-4.89) ng/ml, p = 0.013 and 0.16+/-1.02 vs. 0.06+/-0.91, p = 0.039; respectively). Cortisol was higher (p = 0.002) in patients (399.14+/-160.5 nmol/L vs. 279.8+/-130.1 nmol/L), whereas no differences were found for other hormone axes. In patients, elevations in GH and IGF-1 and decreases in thyroid-stimulating hormone, free triiodothyronine and testosterone (in men) were associated with severity of impairments (Independence scale, Functional score, Total Functional Capacity, Total Motor score, Behavioral score). Only GH was independently associated with body mass index (beta = -0.26, p = 0.001).
  
  
  CONCLUSIONS
  Our data suggest that the thyrotropic and in men gonadotropic axes are altered in HD according to the severity of the disease. The somatotropic axis is overactive even in patients with early disease, and could be related to the weight loss seen in HD patients.
authors.affiliation: Centre de référence maladie de Huntington, AP-HP, Groupe Henri-Mondor Albert-Chenevier/Pitié Salpétrière, Créteil, Paris, France.
authors.forename: Nadine
authors.initials: N
authors.lastname: Saleh
authors.forename: Stéphane
authors.initials: S
authors.lastname: Moutereau
authors.forename: Alexandra
authors.initials: A
authors.lastname: Durr
authors.forename: Pierre
authors.initials: P
authors.lastname: Krystkowiak
authors.forename: Jean-Philippe
authors.initials: JP
authors.lastname: Azulay
authors.forename: Christine
authors.initials: C
authors.lastname: Tranchant
authors.forename: Emmanuel
authors.initials: E
authors.lastname: Broussolle
authors.forename: Françoise
authors.initials: F
authors.lastname: Morin
authors.forename: Anne-Catherine
authors.initials: AC
authors.lastname: Bachoud-Lévi
authors.forename: Patrick
authors.initials: P
authors.lastname: Maison