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

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

Collaborators (220)

Shoulson I, Young A, Kieburtz K, Kayson E, Zhao H, Shinaman MA, Romer M, Biglan K, Hersch S, Penney J, Marder K, Paulsen J, Quaid K, Siemers E, Tanner C, Mallonee W, Palmer D, Suter G, Dubinsky R, Gronseth G, Schimke N, Gray C, Nance M, Bundlie S, Radtke D, Kostyk S, Paulson GW, Thomas K, Stepanov N, Baic C, Caress J, Walker F, Hunt V, Chouinard S, Rouleau G, Poiffaut H, Rioux B, Testa C, Greenamyre T, Harrison J, Corey-Bloom J, Song D, Peavy G, Goldstein J, Paulsen J, Paulson H, Rodnitzky RL, Mikos A, Reese B, Stierman L, Williams K, Vining L, Marder K, Louis E, Moskowitz C, Quaid K, Wojcieszek J, Wesson M, Samii A, Bird T, Lipe H, Reynolds N, Blindauer K, Petit J, Como P, Marshall F, Counihan T, Biglan K, Zimmerman C, Hogarth P, Nutt J, Andrews P, Hersch S, Shinobu L, Rosas D, Kaneko Y, Gevorkian S, Sexton P, Caviness J, Adler C, Wheelock V, Richman D, Tempkin T, Wu CK, Fernandez H, Friedman JH, Lannon M, Seeberger L, O'Brien C, Montellano S, Kartha N, Sakurai S, Hickenbottom S, Albin R, Wernette K, Racette B, Perlmutter JS, Good L, Jackson G, Perlman S, Segal S, Carroll R, Carr L, Martin W, Roberts T, Wieler M, Leavitt B, Clarke L, Raymond L, Decolongon J, Popovska V, Almqvist E, Ondo W, Thomas M, Ashizawa T, Jankovic J, Hauser R, Sanchez-Ramos J, Price K, Delgado H, Furtado S, LaFontaine AL, Suchowersky O, Klimek ML, Sethna R, Guttman M, Russell S, Elliott S, Mentis M, Feigin A, Cox M, Shannon B, Percy A, Dure L, Pendley D, Lane J, Harrison M, Rost-Ruffner E, Johnson W, Colcher A, Siderowf A, Matthews M, Jennings D, Marek K, Caplan K, Factor S, Higgins D, Molho E, Nickerson C, Evans S, Brown D, Hobson D, Shelton P, Hobson S, Singer C, Galvez-Jimenez N, Koller W, Martin D, Lyons K, Rodriguez D, Shannon K, Comella C, Jaglin J, Anderson K, Weiner W, Dustin K, Rosenblatt A, Ross C, Pollard D, Saint-Hilaire MH, Novak P, Fink S, Hersh B, Diggin M, Vickers L, Deckel W, Duffy J, Fitzpatrick MJ, Thomson E, Stout J, Calhoun J, Coryel W, Erwin C, Hunt V, Vawter D, Andrews L, Bury D, Leventhal C, Quaid K, Shinaman A, Wexler N, Brocht A, Daigneault S, Gerwitz K, Orme C, Nobel R, Ross V, Slough M, Watts A, Weber J, Weaver C, Julian-Baros E, Young A, Marder K, Foroud T, Gusella J, Housman D, MacDonald M, Myers R, Tanzi R, Fahn S, Conneally M, Tsai WY, Beal F, Housman D, Kawas C, Francoise-Chesselet M, Shults C, Hogarth P, Zhao H.

Author information

Departments of Neurology and Psychiatry, Taub Institute for Research on Alzheimer's Disease and the Aging Brain, Gertrude H. Sergievsky Center, Columbia University, New York, NY 10032, USA.



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


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.


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.


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.

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