Join Us | Latest Articles | Contact

Journal Home

Editorial Board


Submit to this journal

Current issue

Journal of Nutritional Medicine and Diet Care

The Importance of Nutritional Control and Diet Care in Huntington's Disease

Nieves González*

Renal, Vascular and Diabetes Research Laboratory, IIS-Jiménez Díaz Foundation, The Autonomous University of Madrid, Spanish Biomedical Research Network in Diabetes and Associated Metabolic Disorders (CIBERDEM), Madrid, Spain

*Corresponding author: Nieves González, PhD, Renal, Vascular and Diabetes Research Laboratory, IIS-Jiménez Díaz Foundation, The Autonomous University of Madrid, Spanish Biomedical Research Network in Diabetes and Associated Metabolic Disorders (CIBERDEM), Madrid, Spain, Tel: +34-91-550-48-99, E-mail:
J Nutri Med Diet Care, JNMDC-1-007e, (Volume 1, Issue 2), Editorial
Received: August 18, 2015: Accepted: September 26, 2015: Published: September 28, 2015
Citation: González N (2015) The Importance of Nutritional Control and Diet Care in Huntington's Disease. J Nutri Med Diet Care 1:007e
Copyright: © 2015 González N. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

The first descriptions of chorea, from the Greek word "dance", date back to the middle ages. The condition ''Saint Vitus dance" was initially considered a curse, and was named so, because afflicted individuals were "cured" when they touched churches, which enshrined Saint Vitus relics [1]. In 1872, Dr. George Huntington published a report entitled "On chorea", where he characterized the Huntington's disease (HD) as a genetic condition, which specifically causes degeneration in neurons of the striatum, resulting in a triad of symptoms, and also including emotional alterations and motor impairments [2]. The genetic defect underlying this autosomal dominant pathology, is a CAG triplet expansion (≥ 36 repeats), located in exon 1 of the HTT gene, which encodes the huntingtin in protein [3,4]. Aggregates formation in the cytosol and nucleus, leads to a generalized atrophy of the brain due to the presence of the mutated protein (polyQ). This mutated protein presents a modified folding, which produces a dominant effect on gain of function; and although it is widely expressed in brain neurons, and is ubiquitous in the body; its functionality still remains unknown [5].

HD has an estimated prevalence in Europe and the USA of 5-10 cases per 100,000 worldwide [6], and the symptoms begin about halfway through the average human lifespan (30-50 years of age), progressive and continuously evolving (15-20 years) until the end of the disease, which coincides with the end of life by dementia and death, or in some cases, suicide [7,8]. Despite the fact that scientific community has performed therapeutic advances in slowing the disease progression down and relieved suffering in the patients, a definitive treatment has not emerged yet [9].

Although HD is considered primarily a neurological disorder, in addition to neuronal degeneration and motor modifications, the patient has to face a drastic weight loss-proportional to the size of the CAG triplet expansion [10]. Low weight and unintentional weight loss (> 10% within the last 3-6 months) are frequent during all phases of the disease and its etiology is multifactorial [11-13]. Moreover, the weight is influenced by severe digestive alterations, dysphagia and eating disorders.

Neurological physicians describe that the first signs of the disease are very mild and may include small changes in personality or character. Forgetfulness, clumsiness and erratic movements of the fingers or toes may be a sign. Often, during these early stages of the disease, individuals do not visit any doctor, and may take several years until a medical diagnosis is made. The disease manifests itself very slowly. If family medical record exits a predictive genetic diagnosis could be done. However, in the absence of genetic follow up because a de novo mutation occurs, the delay in the diagnosis could be longer. The estimated probability that a new penetrant HD allele (≥ 36) could arise, in a male carrying a high normal allele (27-35 CAG repeats) during the paternal transmission due to the instability of the repeats ranges from 1/6,241 to 1/951 [14].

As HD progresses, brief, abrupt, irregular, unpredictable, non-stereotyped movements; in milder cases the patient often shows restlessness and clumsiness. These movements affect various body parts, and interfere with speech, swallowing, posture and gait [15]. Moreover, constant movement increases energy expenditure and requires following a high calorie diet [16]. In advance stages, when oral diet may not provide for the patient's nutritional needs and due to cachexia, enteral feeding must be considered [17]. Not only before the symptoms appear, but also throughout the duration of the disease, nutritional aspects and diet care in individuals diagnosed with HD should be followed, by specific healthcare professionals. Recommendations are provided by limited guidelines, which are focused on specific instructions in the early, middle and advance stages of the disease, for patients, families and caregivers.

The research related to HD's diet is limited; however dietary interventions in HD asymptomatic carriers have been described in a close connection with the subsequent onset of the disease. One observational study has reported that high consumption of dairy products has a two-fold increased risk of phenoconversion [18]. Also, higher baseline uric acid levels are associated with slower HD progression, which potentially suggests that rich purines food could be beneficial in an HD's diet [19]. Moreover, in a global perspective, Mediterranean diet does not have impact on HD onset, although higher caloric intake is associated with a risk of phenoconversion [20]. On the other hand, recently, a Spanish multicenter dietary intake study has reported that an adequate dietary intake prevents against weight loss in patients with advanced HD, but it is not associated with better functional state [21]. However, the information related to diet and HD's phenoconversion is limited, being not definitive enough to allow a specific conclusion. The small number of studies and their no replications lead us to carefully consider those outcomes.

Metabolic aspects such as glucose and lipid profiles, control weight, nutrient absorption could represent key factors in the quality of life for patients with HD, being heavily influential in morbidity and mortality. For example: length of hospital care, severity of the disease and efficiency of the pharmacological therapies; representing clinical relevance for its potential contribution to the symptoms, treatment and influence on the course of the disease. Studies on diet and energy expenditure in pre-manifest HD carriers may provide knowledge for interventions to modify specific components of the diet that may delay the onset of the disease. Additionally, research should include the study on the patient status after the onset, when the nutritional control and diet care become even more essential.

The ongoing and future research should be focused on a treatment approach, which would include peripheral alterations and also central pathologies in HD, setting up the basis for a more effective therapeutic strategy; the maintenance of a healthy nervous system is closely linked to the metabolic health.

The support of the importance of nutritional control and diet care in Huntington's disease will be provided by the Journal of Nutritional Medicine and Diet Care. The journal will constitute a platform for the interaction between the experts around the world, and will aim at spreading scientific discoveries, and major milestones in the field not only of HD, but also of a wide range of neurological syndromes (eg. Alzheimer disease, Down syndrome and Friedreich ataxia), which evolve in parallel with the metabolic alterations.


The author thanks Persan Farma (Ref.:12224/3) for the collaboration to the manuscript, and Marcin Koza for proofreading the manuscript.

  1. Vale TC, Cardoso F (2015) Chorea: A Journey through History. Tremor Other Hyperkinet Mov (N Y) 5.

  2. Huntington G (1872) On Chorea. Medical and Surgical Reporter 26: 317-321.

  3. MacDonald ME, Novelletto A, Lin C, Tagle D, Barnes G, et al. (1992) The Huntington's disease candidate region exhibits many different haplotypes. Nat Genet 1: 99-103.

  4. HD Collaborative Researched Group (1993) A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes. Cell 72: 971-983.

  5. Davies SW, Turmaine M, Cozens BA, DiFiglia M, Sharp AH, et al. (1997) Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation. Cell 90: 537-548.

  6. Landles C, Bates GP (2004) Huntingtin and the molecular pathogenesis of Huntington's disease. Fourth in molecular medicine review series. EMBO Rep 5: 958-963.

  7. Sharp AH, Loev SJ, Schilling G, Li SH, Li XJ, et al. (1995) Widespread expression of Huntington's disease gene (IT15) protein product. Neuron 14: 1065-1074.

  8. Hubers AA, van Duijn E, Roos RA, Craufurd D, Rickards H, et al. (2013) Suicidal ideation in a European Huntington's disease population. J Affect Disord 151: 248-258.

  9. Shannon KM, Fraint A (2015) Therapeutic advances in Huntington's Disease. Mov Disord 30: 1539-1546.

  10. Aziz NA, van der Burg JM, Landwehrmeyer GB, Brundin P, Stijnen T; EHDI Study Group, et al. (2008) Weight loss in Huntington disease increases with higher CAG repeat number. Neurology 71: 1506-1513.

  11. Petersén A, Gil J, Maat-Schieman ML, Bjorkqvist M, Tanila H, et al. (2005) Orexin loss in Huntington's disease. Hum Mol Genet 14: 39-47.

  12. Popovic V, Svetel M, Djurovic M, Petrovic S, Doknic M, et al. (2004) Circulating and cerebrospinal fluid ghrelin and leptin: potential role in altered body weight in Huntington's disease. Eur J Endocrinol 151: 451-455.

  13. Lin MT, Beal MF (2006) Mitochondrial dysfunction and oxidative stress in neurodegenerative diseases. Nature 443: 787-795.

  14. Hendricks AE, Latourelle JC, Lunetta KL, Cupples LA, Wheeler V, et al. (2009) Estimating the probability of de novo HD cases from transmissions of expanded penetrant CAG alleles in the Huntington disease gene from male carriers of high normal alleles (27-35 CAG). Am J Med Genet A 149A: 1375-1381.

  15. Walker FO (2007) Huntington's disease. Lancet 369: 218-228.

  16. Trejo A, Boll MC, Alonso ME, Ochoa A, Velásquez L (2005) Use of oral nutritional supplements in patients with Huntington's disease. Nutrition 21: 889-894.

  17. Kreymann KG, Berger MM, Deutz NE, Hiesmayr M, Jolliet P, et al. (2006) ESPEN Guidelines on Enteral Nutrition: Intensive care. Clin Nutr 25: 210-223.

  18. Buruma OJ, Van der Kamp W, Barendswaard EC, Roos RA, Kromhout D, et al. (1987) Which factors influence age at onset and rate of progression in Huntington's disease? J Neurol Sci 80: 299-306.

  19. Auinger P, Kieburtz K, McDermott MP (2010) The relationship between uric acid levels and Huntington's disease progression. Mov Disord 25: 224-228.

  20. Marder K, Gu Y, Eberly S, Tanner CM, Scarmeas N, et al. (2013) Relationship of Mediterranean diet and caloric intake to phenoconversion in Huntington disease. JAMA Neurol 70: 1382-1388.

  21. Cubo E, Rivadeneyra J, Armesto D, Mariscal N, Martinez A, et al. (2015) Relationship between Nutritional Status and the Severity of Huntington's Disease. A Spanish Multicenter Dietary Intake Study. J Huntingtons Dis 4: 78-85.

International Journal of Anesthetics and Anesthesiology (ISSN: 2377-4630)
International Journal of Blood Research and Disorders   (ISSN: 2469-5696)
International Journal of Brain Disorders and Treatment (ISSN: 2469-5866)
International Journal of Cancer and Clinical Research (ISSN: 2378-3419)
International Journal of Clinical Cardiology (ISSN: 2469-5696)
Journal of Clinical Gastroenterology and Treatment (ISSN: 2469-584X)
Clinical Medical Reviews and Case Reports (ISSN: 2378-3656)
Journal of Dermatology Research and Therapy (ISSN: 2469-5750)
International Journal of Diabetes and Clinical Research (ISSN: 2377-3634)
Journal of Family Medicine and Disease Prevention (ISSN: 2469-5793)
Journal of Genetics and Genome Research (ISSN: 2378-3648)
Journal of Geriatric Medicine and Gerontology (ISSN: 2469-5858)
International Journal of Immunology and Immunotherapy (ISSN: 2378-3672)
International Journal of Medical Nano Research (ISSN: 2378-3664)
International Journal of Neurology and Neurotherapy (ISSN: 2378-3001)
International Archives of Nursing and Health Care (ISSN: 2469-5823)
International Journal of Ophthalmology and Clinical Research (ISSN: 2378-346X)
International Journal of Oral and Dental Health (ISSN: 2469-5734)
International Journal of Pathology and Clinical Research (ISSN: 2469-5807)
International Journal of Pediatric Research (ISSN: 2469-5769)
International Journal of Respiratory and Pulmonary Medicine (ISSN: 2378-3516)
Journal of Rheumatic Diseases and Treatment (ISSN: 2469-5726)
International Journal of Sports and Exercise Medicine (ISSN: 2469-5718)
International Journal of Stem Cell Research & Therapy (ISSN: 2469-570X)
International Journal of Surgery Research and Practice (ISSN: 2378-3397)
Trauma Cases and Reviews (ISSN: 2469-5777)
International Archives of Urology and Complications (ISSN: 2469-5742)
International Journal of Virology and AIDS (ISSN: 2469-567X)
More Journals

Contact Us

ClinMed International Library | Science Resource Online LLC
3511 Silverside Road, Suite 105, Wilmington, DE 19810, USA


Get Email alerts
Creative Commons License
Open Access
by ClinMed International Library is licensed under a Creative Commons Attribution 4.0 International License based on a work at
Copyright © 2017 ClinMed International Library. All Rights Reserved.