Research Article| Volume 279, ISSUE 1-2, P26-29, April 15, 2009

Do patients with amyotrophic lateral sclerosis (ALS) have increased energy needs?

Published:February 02, 2009DOI:


      Background and aims

      Nutritional status is a prognostic factor for survival in amyotrophic lateral sclerosis (ALS) patients. We investigated the contribution of some of the components contributing to resting energy expenditure (REE) in order to determine whether potentially higher energy needs should be considered for these patients.


      Thirty three ALS patients and 33 age- and gender-matched healthy controls participated. REE was measured by an open-circuit indirect calorimeter, body composition by dual energy X-ray absorptiometry, and estimated caloric intake by 7-day food records.


      Patients had lower body mass indices and lower lean body mass (LBM) than healthy controls. REE values (as a percentage of predicted) was similar but increased when normalized by LBM (P<0.001). LBM and REE decreased while REE/LBM increased in ten patients who were reassessed 6 months later. A model for predicting measured REE was constructed based on the different components, with 86% prediction of its variability.


      ALS is associated with increased REE. Various factors, such as poor caloric intake and mechanical ventilation, may mask this tendency. All the above parameters need to be considered during nutritional intervention to prevent additional muscle loss.


      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online access
      One-time access price info
      • For academic or personal research use, select 'Academic and Personal'
      • For corporate R&D use, select 'Corporate R&D Professionals'


      Subscribe to Journal of the Neurological Sciences
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect


        • Desport J.C.
        • Preux P.M.
        • Truong T.C.
        • Vallat J.M.
        • Sautereau D.
        • Couratier P.
        Nutritional status is a prognostic factor for survival in ALS patients.
        Neurology. 1999; 53: 1059-1063
        • Kasarskis E.J.
        • Berryman S.
        • Vanderleest J.G.
        • Schneider A.R.
        • McClain C.J.
        Nutritional status of patients with amyotrophic lateral sclerosis: relation to the proximity of death.
        Am J Clin Nutr. 1996; 63: 130-137
        • Desport J.C.
        • Preux P.
        • Magy L.
        • et al.
        Factors correlated with hypermetabolism in patients with amyotrophic lateral sclerosis.
        Am J Clin Nutr. 2001; 74: 328-334
        • Desport J.C.
        • Torny F.
        • Lacoste M.
        • Preux P.M.
        • Couratier P.
        Hypermetabolism in ALS: correlations with clinical and paraclinical parameters.
        Neurodegener Dis. 2005; 2: 202-207
        • Dupuis I.
        • Oudart H.
        • Rene F.
        • Gonzalez de Aguilar J.L.
        • Loeffler J.P.
        Evidence for defective energy homeostasis in amyotrophic lateral sclerosis: benefit of a high-energy diet in transgenic mouse model.
        PNAS. 2004; 101: 11159-11164
        • Leigh P.N.
        • Ray-Chaudhuri K.
        Motor neuron disease.
        J Neurol Neurosurg Psychiatry. 1994; 57: 886-896
        • Keys A.
        • Brozek J.
        • Hens A.
        • Mickelson O.
        • Taylor H.L.
        The biology of human starvation.
        University of Minnesota Press, Minneapolis, MN1950
        • Vaisman N.
        • Clarke R.
        • Pencharz P.B.
        Nutritional rehabilitation increases resting energy expenditure without affecting protein turnover in patients with cystic fibrosis.
        J Pediatr Gastroenterol Nutr. 1991; 13: 383-390
        • Wang Z.
        • Heshka S.
        • Gallagher D.
        • Boozer C.N.
        • Kotler D.P.
        • Heymsfield S.B.
        Resting energy expenditure-fat free-mass relationship: new insights provided by body composition modeling.
        Am J Physiol Endocrinol Metabol. 2000; 279: E539-545
        • Echaniz-Laguna A.
        • Zoll J.
        • Ribera F.
        • Tranchant C.
        • Warter J.M.
        • Lonsdorfer J.
        • Lampert E.
        Mitochondrial respiratory chain function in skeletal muscle of ALS patients.
        Ann Neurol. 2002; 52: 623-627
        • Martin L.J.
        Mitochondriopathy in Parkinson disease and amyotrophic lateral sclerosis.
        J Neuropathol Exp Neurol. 2006; 65: 1103-1110
        • Brooks B.R.
        • Miller R.G.
        • Swash M.
        • Munsat T.L.
        El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis.
        in: Amyotroph. Later. Scler. And other Motor neuron Disord.1. 2000: 293-299
        • Cedarbaum J.M.
        • Stambler N.
        • Malta E.
        • Fuller C.
        • Hilt D.
        • Thurmond B.
        • Nakanishi A.
        The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. BDNF ALS Study Group (Phase III).
        J Neurol Sci. 1999; 169: 13-21
        • Ferrannini E.
        The theoretical bases of indirect calorimetry: a review.
        Metabolism. 1988; 37: 287-301
        • Lukaski H.C.
        Methods for assessment of human body composition: traditional and new.
        Am J Clin Nutr. 1987; 46: 537-556
        • Sherman M.S.
        • Pillai A.
        • Jackson A.
        • Heiman-Patterson T.
        Standard equations are not accurate in assessing resting energy expenditure in patients with amyotrophic lateral sclerosis.
        JPEN. 2004; 28: 442-446
        • Harris J.A.
        • Benedict F.G.
        A biometric study of basal metabolism in man.
        Carnegie Institute of Washington, Washington, DC1919 (Publication number 297)
        • Vaisman N.
        • Katzenellenbogen S.
        • Nevo Y.
        Increased resting energy expenditure in subjects with Emery-Dreifuss muscular dystrophy.
        Neuromuscul Dis. 2004; 14: 142-146
        • Kasarskis E.J.
        • Berryman S.
        • Vanderleest J.G.
        • Schneider A.R.
        • McClain C.J.
        Nutritional status of patients with amyotrophic lateral sclerosis: relation to the proximity of death.
        Am J Clin Nutr. 1996; 63: 130-137
        • Shimizu T.
        • Hayashi H.
        • Tanabe H.
        Energy metabolism of ALS patients under mechanical ventilation and tube feeding.
        Clin Neurol. 1991; 31: 255-259
        • Nau K.L.
        • Bromberg M.B.
        • Forshew D.A.
        • Katch V.L.
        Individuals with amyotrophic lateral sclerosis are in caloric balance despite losses in mass.
        J Neurol Sci. 1995; 129: 47-49
        • Dupuis L.
        • Gonzalez de Aguilar J.L.
        • Oudart H.
        • de Tapia M.
        • Barbeito L.
        • Loeffler J.P.
        Mitochondria in amyotrophic lateral sclerosis: a trigger and target.
        Neurodegener Dis. 2004; 1: 245-254