TDNet Discover

Background: Huntington’s disease (HD) is an autosomal dominant-inherited neurodegenerative disorder that results in cerebral atrophy leading to characteristic mood, movement, and memory changes that typically present between ages 30 to 50. Memory changes from dementia develop in most patients before age 50. There is currently no cure for HD and current treatments aim to minimize symptoms, improve comfort and quality of life. Unintended weight loss is a hallmark feature of HD and is arguably one of the most important non-neurologic complications of the disease process as it has a direct impact on the patient’s quality of life. A limited number of studies have been conducted to evaluate effective dietary and nutritional changes in patients with HD that may alter the progression of the disease or reduce the burden of unintended weight loss. Objective: The purpose of this research is to identify dietary or nutritional interventions that may alter the progression of the disease with a focus on the ability to reduce the amount of unintended weight loss in the HD population. Methods: A systematic review of the literature pertaining to dietary interventions in HD patients was performed by reviewing available abstracts through PubMed, ProQuest, and EBSCOhost. Final article selection was made through group consensus after review of full texts and adherence to established inclusion and exclusion criteria. A total of eight articles were selected and analyzed according to the National Heart, Lung and Blood Institute quality assessment tool. Data from these articles was extracted and analyzed based on dietary intervention and their corresponding efficacy on reducing or preventing unintended weight loss. Three articles focused on the Mediterranean diet in HD patients, two looked at the difference in energy expenditure in HD patients versus non-HD patients, one explored a customized diet plan, one looked at oral nutrition supplementation, and the final study explored the effect of standard protein diet versus high protein diet in HD patients. Results: Our literature review resulted in eight studies that compared the efficacy of four different dietary interventions aimed at reducing the severity and progression of HD. Three articles identified an increased metabolic demand in HD. An average activity factor of 0.28 times greater than individuals without HD was found;a 20% increase in caloric intake via supplementation resulted in 87% of patients maintaining a stable weight. Three studies investigated the effect of the Mediterranean diet (MD) on HD patients with a summative conclusion that there is a lack of sufficient evidence that adherence to MD provides a significant benefit to HD patients. One study determined that serum citrulline levels were an inadequate indicator of HD progression, nor did a high protein diet alter the progression of HD. Two studies found that customized supplemental diets in HD patients resulted in weight gain or reduction in weight loss rates. Due to their small sample sizes, additional research with larger cohorts is needed to provide more substantial evidence. Further studies are needed to explore their efficacy as well as their application to patients with advanced stages of HD.