Characteristics Of Clinical Manifestations,Pulmonary Dysfunction And ALSFRS-R Changes In 181 Amyotrophic Lateral Sclerosis Patients

ObjectiveTo explore the characteristics of pulmonary dysfunction and ALSFRS-R changes in amyotrophic lateral sclerosis(ALS)patients.MethodsWe enrolled 181 ALS patients from Feb 2017 to Dec 2018 and collected clinical information including sex,birth date,BMI,site of onset symptom,symptom onset date,diagnosis date,diagnosis level and pulmonary function report.ALSFRS-R was evaluated during hospitalization and △FS was calculated.The patients enrolled from Feb to Jul 2017 were followed in Nov 2018 and Feb 2019 separately.The patients enrolled from Aug 2017 to Dec 2018 were followed every 3 to 6 months by telephone.The information about survive,ALSFRS-R,mechanical ventilation and assistant feeding was collected by follow-up.Patients were separated by sex,site of onset symptom,diagnosis rate and pulmonary function was compared between different groups.Longitudinal study of ALSFRS-R was performed in 65 patients who had been followed consecutive 6 months.ResultsThere were 110 male patients and 71 female patients;the male:female ratio was 1.55:1.The mean age at symptom onset was 53.6±10.5 years old,the mean time from onset to diagnosis was 14.6±14.4 months.Except for respiratory subscore between T1 and T0,ALSFRS-R and its subscore decreased significantly as time went on.Except for Δ FS of motor function between T0-T1 and T1-T2,ΔFS and ΔFS of subscore were stable in 6 months.ΔFS of bulbar function andΔFS of motor function were significantly diferent between different site of onset.As for pulmonary dysfunction,66.9%patients had normal pulmonary function result,and 27.6%patients had restrictive pulmonary dysfunction.The pulmonary function includingFVC%,FEV1%,PEF%,MV%was significantly low in bulbar onset group.FVC%,PEF%and FEV1/FVC were significantly differently among different diagnosis rate.Logistics analysis revealed that site of onset was related to pulmonary dysfunction.The positive rate of respiratory dysfunction symptom and MVV%decline was significantly different.Kaplan-Meier analysis revealed the time of symptom onset to diagnosis,FVC%,FEV1%,MVV%,PEF%,A FS were prognosis factor.In the Cox multivariate model,time from onset to diagnosis and PEF%were significantly related to survival.Conclusion1.ALSFRS-R gradually decreases as time went on,and Δ FS remain unstable within 6 months.2.The main pulmonary dysfunction is restrictive type,and site of onset is related to pulmonary dysfunction.MVV%is contribute to reveal subclinical pulmonary dysfunction.3.The time from onset to diagnosis and PEF% are predictor of survival.