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Growth hormone response to arginine test differentiates between two subgroups of Huntington's disease patients
  1. Elena Salvatore1,
  2. Carlo Rinaldi1,
  3. Tecla Tucci1,
  4. Luigi Di Maio1,
  5. Carolina Di Somma2,
  6. Silvia Savastano3,
  7. Gaetano Lombardi3,
  8. Alessandro Filla1,
  9. Annamaria Colao3,
  10. Giuseppe De Michele1
  1. 1Dipartimento di Scienze Neurologiche, Università degli Studi di Napoli ‘Federico II’, Naples, Italy
  2. 2IRCCS Fondazione SDN, Istituto di Ricerca Diagnostica e Nucleare, Naples, Italy
  3. 3Dipartimento di Endocrinologia ed Oncologia Molecolare e Clinica, Università degli Studi di Napoli, ‘Federico II’, Naples, Italy
  1. Correspondence to Dr Giuseppe De Michele, Dipartimento di Scienze Neurologiche, Università degli Studi di Napoli Federico II, Via Pansini 5, 80131, Naples, Italy; demichel{at}unina.it

Abstract

Objective Huntington's disease (HD) is an autosomal dominant disorder characterised by motor, cognitive and psychiatric disturbances. Several studies have demonstrated that hypothalamic dysfunction is part of the phenotypic spectrum. The aim of the study was to evaluate the growth hormone (GH) response to arginine infusion in a cohort of HD patients, to search for an in vivo biomarker of hypothalamic dysfunction.

Methods The authors investigated 17 HD patients and 17 age-, sex- and BMI-matched healthy controls. Clinical assessment of patients was performed using the Unified Huntington's Disease Rating Scale motor section and total function capacity. Metabolic and endocrine investigations included total, LDL and HDL cholesterol, basal insulin, GH, insulin-like growth factor 1 (IGF-1), SD Score IGF-1 (SDS IGF-1) and the GH response to arginine stimulation.

Results HD patients showed lower plasma IGF-1 and SDS IGF-1 levels and a higher baseline GH in comparison with control subjects. The arginine test induced a normal GH peak in nine patients (53%; Arg+), whereas the response was absent in the remaining eight (47%; Arg–). Arg+ and Arg– also showed two distinct endocrine/metabolic profiles with differences in insulin and lipid metabolism.

Conclusion It remains to be clarified if these two subgroups of patients, according to the GH response to arginine, correspond to different disease stages or to different patterns of neurodegeneration.

  • Huntington's disease
  • neuroendocrinology
  • growth hormone
  • arginine test

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Footnotes

  • Competing interests None.

  • Patient consent Obtained.

  • Ethics approval Ethics approval was provided by the Ethics Committee of the University of Federico II, Naples, Italy.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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