摘要INTRODUCTIONAmyotrophic lateral sclerosis (ALS) is characterized by the progressive degeneration of upper and lower motor neurons.Typical clinical features of ALS are limb paralysis,muscle atrophy,dysphagia,dysarthria,shortness of breath,and respiratory failure.Approximately 90％ of ALS cases are classified as sporadic ALS,the remaining 10％ are classified as familial.[1,2] Researchers have found that the survival of ALS patients is related to several factors,including clinical phenotype,age at onset,sex,early presence of respiratory failure,and treatment with riluzole.We recently found that there is a potential linear relationship in ALS between serum lactate and motor deterioration,and that slower lactate elimination rate might be associated with faster disease progression.[3] In addition,several studies have reported that nutritional status is closely related to the survival time of ALS patients,and there exists a U-shaped association between patients' body mass index (BMI) and mortality.[1,4] The main cause of malnutrition (BMI ＜18.5 kg/m2) in ALS patients is an imbalance between intake and consumption,and some symptoms,such as dysphagia,can lead to insufficient energy intake.More importantly,recent studies have shown that ALS patients are in states of hypermetabolism.[5,6]