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Neuromuscular
Original research
The hypometabolic state: a good predictor of a better prognosis in amyotrophic lateral sclerosis
  1. Marina Cattaneo1,2,
  2. Pierre Jesus3,4,
  3. Andrea Lizio1,
  4. Philippe Fayemendy4,5,
  5. Nicoletta Guanziroli2,
  6. Ettore Corradi2,
  7. Valeria Sansone1,6,
  8. Letizia Leocani7,8,
  9. Massimo Filippi8,9,
  10. Nilo Riva7,9,
  11. Philippe Corcia10,11,
  12. Philippe Couratier4,12,
  13. Christian Lunetta1
  1. 1 NeuroMuscular Omnicentre (NeMO)—Fondazione Serena Onlus, Milano, Italy
  2. 2 ASST Grande Ospedale Metropolitano Niguarda, Milano, Italy
  3. 3 Nutrition Unit, University Hospital Centre of Limoges, Limoges, France
  4. 4 Inserm UMR 1094, Tropical Neuroepidemiology, University of Limoges Medical Faculty, Limoges, France
  5. 5 Nutrition Unit, Limoges, France
  6. 6 Department of Biomedical Sciences of Health, University of Milan, Milano, Italy
  7. 7 Neurorehabilitation Unit, San Raffaele Hospital, Milano, Italy
  8. 8 Vita-Salute San Raffaele University, Milano, Italy
  9. 9 Neurology Unit, San Raffaele Hospital, Milano, Italy
  10. 10 ALS Center, University Hospital of Tours, Tours, France
  11. 11 Inserm Unit 1253, iBrain, Tours, France
  12. 12 Centre de reference maladies rares SLA et autres maladies du neurone moteur, Centre Hospitalier Universitaire de Limoges, Limoges, France
  1. Correspondence to Dr Christian Lunetta, NeuroMuscular Omnicentre (NeMO) - Fondazione Serena Onlus, Milano, Italy; christian.lunetta{at}centrocliniconemo.it

Abstract

Background Malnutrition and weight loss are negative prognostic factors for survival in patients with amyotrophic lateral sclerosis (ALS). However, energy expenditure at rest (REE) is still not included in clinical practice, and no data are available concerning hypometabolic state in ALS.

Objective To evaluate in a referral cohort of patients with ALS the prevalence of hypometabolic state as compared with normometabolic and hypermetabolic states, and to correlate it with clinical phenotype, rate of progression and survival.

Design We conducted a retrospective study examining REE measured by indirect calorimetry in patients with ALS referred to Milan, Limoges and Tours referral centres between January 2011 and December 2017. Hypometabolism and hypermetabolism states were defined when REE difference between measured and predictive values was ≤−10% and ≥10%, respectively. We evaluated the relationship between these metabolic alterations and measures of body composition, clinical characteristics and survival.

Results Eight hundred forty-seven patients with ALS were recruited. The median age at onset was 63.79 years (IQR 55.00–71.17). The male/female ratio was 1.26 (M/F: 472/375). Ten per cent of patients with ALS were hypometabolic whereas 40% were hypermetabolic. Hypometabolism was significantly associated with later need for gastrostomy, non-invasive ventilation and tracheostomy placement. Furthermore, hypometabolic patients with ALS significantly outlived normometabolic (HR=1.901 (95% CI 1.080 to 3.345), p=0.0259) and hypermetabolic (HR=2.138 (95% CI 1.154 to 3.958), p=0.0157) patients.

Conclusion Hypometabolism in ALS is not uncommon and is associated with slower disease progression and better survival than normometabolic and hypermetabolic subjects. Indirect calorimetry should be performed at least at time of diagnosis because alterations in metabolism are correlated with prognosis.

Data availability statement

Data are available on reasonable request. De-identified database will be shared on reasonable request for 2 years after publication by contacting the corresponding author at the following email: christian.lunetta@centrocliniconemo.it.

https://doi.org/10.1136/jnnp-2021-326184

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    Data availability statement

    Data are available on reasonable request. De-identified database will be shared on reasonable request for 2 years after publication by contacting the corresponding author at the following email: christian.lunetta@centrocliniconemo.it.

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    Footnotes

    • Contributors MC: drafting/revising the manuscript, study concept and acquisition, analysis and interpretation of the data. PJ: drafting/revising the manuscript, study concept and acquisition and interpretation of the data. AL: drafting/revising the manuscript and statistical analysis of the data. PF: drafting/revising the manuscript and acquisition and interpretation of the data. NG: drafting/revising the manuscript and acquisition and interpretation of the data. EC: drafting/revising the manuscript and interpretation of the data. VS: drafting/revising the manuscript and interpretation of the data. LL: drafting/revising the manuscript and interpretation of the data. MF: drafting/revising the manuscript, interpretation of the data and study supervisor. NR: drafting/revising the manuscript, study concept and acquisition and interpretation of the data. PCor: drafting/revising the manuscript, study concept, interpretation of the data and study supervisor. PCou: drafting/revising the manuscript, study concept, interpretation of the data and study supervisor. CL: drafting/revising the manuscript, study concept, interpretation of the data and study supervisor.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests AL received compensation for occasional scientific consulting from Italfarmaco. PF, NG and EC have nothing to disclose. VS participates in Advisory Boards or Teaching activities for Biogen, Roche, Avexis, PTC, Santhera, Sarepta, Dyne. LL received honoraria for consulting services from Merck, Roche, Novartis and for speaking activities from Teva; research support from Merck, Biogen, Novartis; travel support from Merck, Roche, Biogen. MF is Editor-in-Chief of the Journal of Neurology; received compensation for consulting services and/or speaking activities from Bayer, Biogen Idec, MerckSerono, Novartis, Roche, Sanofi Genzyme, Takeda and Teva Pharmaceutical Industries; and receives research support from Biogen Idec, Merck-Serono, Novartis, Roche, Teva Pharmaceutical Industries, Italian Ministry of Health, Fondazione Italiana Sclerosi Multipla and ARiSLA (Fondazione Italiana di Ricerca per la SLA). NR, PCor and PCou have nothing to disclose. CL served on a scientific advisory board for Mitsubishi Tanabe Pharma Europe, Cytokinetics, Neuraltus and Italfarmaco and has received funds from ARISLA and Ministry of Health (CCM2011).

    • Provenance and peer review Not commissioned; externally peer reviewed.