Bulbar involvement time in spinal-onset amyotrophic lateral sclerosis: Effect on survival / 西安交通大学学报(医学版)

ABSTRACT

Objective: To explore the effect of bulbar involvement time on survival time of patients with spinal-onset amyotrophic lateral sclerosis (ALS). Methods: We followed up 168 patients with spinal-onset ALS admitted to our hospital from January 2, 2011 to December 31, 2017 until December 31, 2018. Univariate and multivariate analyses were performed to evaluate the affecting factors of the ALS patients' survival time. Kaplan-Meier analysis was made to evaluate the effects of bulbar involvement time on survival time. Results: COX multivariate analysis showed that the risk of death in age-onset <55 y patients was 0.72 times that in age-onset ≥55 y (P=0.09), the risk of death in diagnosis delay time <10.98 m patients was 2.64 times that ≥10.98 m (P<0.001); the risk of death in bulbar involvement time ≥11.5 m and bulbar uninvolvement was 0.30 and 0.32 times respectively that bulbar involvement time <11.5 m (P<0.001). Kaplan-Meier analysis showed differences among bulbar involvement time <11.5 m, ≥11.5 m and bulbar uninvolvement groups (median survival time 20.37 m vs. 40.6 m vs. 39.60 m, Test statistic =39.96, P<0.001). The 2-year, 3-year and 5-year survival rates were 32.17%, 10.80% and 0%, respectively, in bulbar involvement time <11.5 m patients; 89.20%, 57.24% and 10.53% in bulbar involvement time ≥11.5 months patients; and 62.16%, 38.39% and 10.53% in bulbar uninvolvement patients. Conclusion: Similar to the diagnosis delay time and whether to have taken riluzole, the occurrence of bulbar involvement at 11.5 month after onset was an independent risk factor affecting survival time in spinal-onset ALS. The median survival time in patients with bulbar involvement time <11.5 months was significantly shorter than that in patients with bulbar involvement time ≥11.5 months and bulbar uninvolvement.