• Edward J Kasarskis, Marta S Mendiondo, Dwight E Matthews, Hiroshi Mitsumoto, Rup Tandan, Zachary Simmons, Mark B Bromberg, Richard J Kryscio, and ALS Nutrition/NIPPV Study Group.
• Department of Neurology (EJK), the Graduate Center for Nutrition (EJK), and the Graduate Center for Toxicology (EJK); the Department of Biostatistics, College of Public Health (MSM); the Sanders Brown Center on Aging (MSM and RJK); and the Department of Statistics, College of Arts and Science (RJK), University of Kentucky, Lexington, KY; Neurology Service, Lexington VA Medical Center, Lexington, KY (EJK); the Departments of Chemistry and Medicine (DEM) and Neurology (RT), University of Vermont, Burlington, VT; the Department of Neurology, Columbia University, New York, NY (HM); the Department of Neurology, Pennsylvania State University, Hershey, PA (ZS); and the Department of Neurology, University of Utah, Salt Lake City, UT (MBB).
• Am. J. Clin. Nutr. 2014 Apr 1;99(4):792-803.

BackgroundPatients with amyotrophic lateral sclerosis (ALS) experience progressive limb weakness, muscle atrophy, and dysphagia, making them vulnerable to insufficient energy intake. Methods to estimate energy requirements have not been devised for this patient group.ObjectiveThe goal was to develop equations to estimate energy requirements of ALS patients.DesignWe enrolled 80 ALS participants at varying stages of their illness and studied them every 16 wk over 48 wk. At each time, we determined total daily energy expenditure (TDEE) in the home setting over a 10-d period by using the doubly labeled water method. We then developed statistical models to estimate TDEE by using factors easily obtained during a routine clinical visit.ResultsThe most practical TDEE models used the Harris-Benedict, Mifflin-St Jeor, or Owen equations to estimate resting metabolic rate (RMR) and 6 questions from the revised ALS Functional Rating Scale (ALSFRS-R) that relate to physical activity. We developed a Web-based calculator to facilitate its use. In the research setting, measuring body composition with bioelectrical impedance spectroscopy enabled the estimation of RMR with the Rosenbaum equation and the same 6 questions from the ALSFRS-R to estimate TDEE. By using these models, the estimate of TDEE for nutritional maintenance was ±500 kcal/d across the spectrum of ALS progression.ConclusionsOur results emphasize the importance of physical function and body composition in estimating TDEE. Our predictive equations can serve as a basis for recommending placement of a feeding gastrostomy in ALS patients who fail to meet their energy requirements by oral intake.

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