2024 Volume 41 Issue 4 Pages 514-517
Nutritional intervention is increasingly recognized as a potential disease–modifying therapy in amyotrophic lateral sclerosis (ALS), particularly for patients with rapid disease progression. This highlights the importance of identifying high–risk patients early. Hypermetabolism in ALS patients is typically associated with a poorer prognosis. However, contrasting findings from ALS model mice, where increased β–oxidation resulted in higher resting energy expenditure and extended survival. Considering the ‘Fuel switch’ phenomenon, where starvation leads to a primary focus on fat metabolism, the impact of hypermetabolism on survival may differ based on metabolic pathways.
In our study, we explored the relationship between hypermetabolism and survival in ALS patients, taking into account their nutritional status. Our findings reveal that hypermetabolism's effect on survival varies with nutritional status : it is generally linked to shorter survival in well–nourished patients but correlates with longer survival in malnourished ones. These results, highlighting the potential protective role of fat metabolism, led us to investigate the relationship between cholesterol levels and ALS prognosis. Contrary to previous studies that linked low low–density lipoprotein cholesterol (LDL) levels with reduced survival and considered high–density lipoprotein cholesterol (HDL) levels non–influential, our study indicates that high HDL levels in both sexes and low LDL levels in women might be associated with poorer prognosis.
Moreover, we assessed the impact of oral calorie intake on ALS prognosis. While the Shimizu formula for total energy expenditure (TEE) is a recognized standard, our findings suggest that actual oral intake relative to ideal body weight (IBW) is a more critical indicator of nutritional status and survival than the gap between TEE and actual intake. Based on this result, percutaneous endoscopic gastrostomy may need to be considered for patients whose oral intake does not meet TEE and is below 25 kcal/kgIBW.
Our study emphasizes the intricate relationship between metabolism, nutritional status, and ALS progression, offering valuable insights for patient management and treatment strategies.
