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Background: Mitochondrial dysfunction plays an important role in the pathogenesis of amyotrophic lateral sclerosis (ALS).We aimed to demonstrate mitochondrial dysfunction in ALS using a lactate stress test and to examine the relationship between mitochondrial dysfunction with motor deterioration.Methods: We enrolled 116 patients and observed clinical variables, including the survival state.Results: Patients with a rapid slope of revised ALS functional rating scales (ALSFRS-r) (＞20 U/year) exhibited the slowest elimination rate (median-4.67 × 10 3 mmol.L-1.min-1, coefficient of variation, 590.15％), the shortest duration (0.63 ± 0.28 years) and the worst ALSFRS-r (32.59 ± 4.93).Patients with a moderate slope of ALSFRS-r (10-20 U/year) showed a moderate elimination rate (median-11.33 × 10 3 mmol.L-1.min-1, coefficient of variation, 309.89％), duration (1.16 ± 0.45 years), andALSFRS-r (34.83 ± 6.11).The slower progressing (＜ 10 U/year group) patients exhibited a rapid elimination rate (median:-12.00 × 10-3 mmol·L-1·min-1, coefficient of variation: 143.08％), longer duration (median: 3 years, coefficient of variation: 193.33％), and adequate ALSFRS-r values (39.58 ± 9.44).Advanced-phase ALS patients also showed slower elimination rate (ER, quartiles-17.33,-5.67, 4.00) and worse ALSFRS-r (34.88 ± 9.27), while early-phase patients showed a more rapid ER (quartiles-25.17,-11.33,-3.50) and betterALSFRS-r (39.28 ± 7.59).These differences were statistically significant.Multiple linear regression analysis revealed strong direct associations among ER, ALSFRS-r slope (standard beta =0.33, P =0.007), and forced vital capacity (predict ％) (standard beta =-0.458, P =0.006, adjusted for ALSFRS-r, course and onset region).However, the data obtained from 3 years of follow-up showed no statistically significant difference in the survival rates between the most rapid and slowest ER groups.Conclusion: There is a potential linear relationship between ER and motor deterioration in ALS.Slower ER might be associated with faster disease progression.