Dietary intake in adults at risk for Huntington disease: analysis of PHAROS research participants

Neurology. 2009 Aug 4;73(5):385-92. doi: 10.1212/WNL.0b013e3181b04aa2.


Objective: To examine caloric intake, dietary composition, and body mass index (BMI) in participants in the Prospective Huntington At Risk Observational Study (PHAROS).

Methods: Caloric intake and macronutrient composition were measured using the National Cancer Institute Food Frequency Questionnaire (FFQ) in 652 participants at risk for Huntington disease (HD) who did not meet clinical criteria for HD. Logistic regression was used to examine the relationship between macronutrients, BMI, caloric intake, and genetic status (CAG <37 vs CAG > or =37), adjusting for age, gender, and education. Linear regression was used to determine the relationship between caloric intake, BMI, and CAG repeat length.

Results: A total of 435 participants with CAG <37 and 217 with CAG > or =37 completed the FFQ. Individuals in the CAG > or =37 group had a twofold odds of being represented in the second, third, or fourth quartile of caloric intake compared to the lowest quartile adjusted for age, gender, education, and BMI. This relationship was attenuated in the highest quartile when additionally adjusted for total motor score. In subjects with CAG > or =37, higher caloric intake, but not BMI, was associated with both higher CAG repeat length (adjusted regression coefficient = 0.26, p = 0.032) and 5-year probability of onset of HD (adjusted regression coefficient = 0.024; p = 0.013). Adjusted analyses showed no differences in macronutrient composition between groups.

Conclusions: Increased caloric intake may be necessary to maintain body mass index in clinically unaffected individuals with CAG repeat length > or =37. This may be related to increased energy expenditure due to subtle motor impairment or a hypermetabolic state.

MeSH terms

  • Adult
  • Body Mass Index*
  • Brain / metabolism
  • Brain / physiopathology
  • DNA Mutational Analysis
  • Dietary Carbohydrates / metabolism
  • Dietary Fats / metabolism
  • Dietary Proteins / metabolism
  • Eating / genetics*
  • Energy Intake / genetics
  • Energy Metabolism / genetics
  • Feeding Behavior / physiology*
  • Female
  • Genetic Predisposition to Disease / genetics
  • Genetic Testing
  • Humans
  • Huntington Disease / genetics*
  • Huntington Disease / metabolism*
  • Huntington Disease / physiopathology
  • Male
  • Middle Aged
  • Predictive Value of Tests
  • Prospective Studies
  • Risk Factors
  • Surveys and Questionnaires
  • Trinucleotide Repeat Expansion / genetics
  • Weight Loss / genetics*


  • Dietary Carbohydrates
  • Dietary Fats
  • Dietary Proteins