Association between estimated total daily energy expenditure and stage of amyotrophic lateral sclerosis

Abstract

Objective Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease, and nutritional status is one of the major prognostic factors of ALS. The aim of this study was to investigate the relationship between total daily energy expenditure (TDEE) and progression of disease in ALS patients, as well as sex differences in TDEE.

Methods Patients with ALS (N = 370) were diagnosed according to El Escorial criteria and categorized into stage 2, 3, or 4 using a clinical staging system. TDEEs were calculated by summing resting energy expenditure (REE) and physical activity. REE was calculated using equations of Harris–Benedict or Mifflin St. Jeor, and physical activity was calculated using the ALS Functional Rating Scale-Revised, a physical activity coefficient, or adding 30% of REE.

Results TDEE significantly decreased with progression of ALS stage and did not vary by sex. Actual energy intake was lower than TDEE in patients in all stages and lowest in patients with stage 3 ALS. Comparing TDEEs 1 to 5, TDEE 2 decreased with progression of ALS stage and was similar to the average TDEE.

Conclusions The present study suggests that TDEE decreases with progression of ALS, and patients consume insufficient energy compared with required intake at all stages, particularly at stage 3, suggesting that nutrition support should be started at least before stage 3. Additionally, among the five equations for TDEE, TDEE 2 could be the best for evaluating the nutritional status of patients with ALS.