Prognostic Value of Iterative Lung Function Testing in Amyotrophic Lateral Sclerosis (Als)

We hypothesized that, in addition to baseline values, changes in lung function values measured during the 6 first mo of follow-up may be prognostic in ALS. Methods 113 patients from the Strasbourg ALS Centre completed follow-up of slow vital capacity (VC), maximal inspiratory pressure (MIP) and sniff nasal pressure (SNIP) till death. The best of either MIP or SNIP (called S/MIP) was used. Parameters were measured every 3 mo. Results Patients’ characteristics are presented in Table 1. Mean survival was 20 (+/-19) months. Baseline values were related to survival. A VC 2 O had a PPV of survival 60 cmH 2 O was associated with survival >6 mo in 97% of patients. Considering changes in VC, a decrease >40% at 3 mo had a PPV of survival 40% at 6 mo had a PPV of survival 6 mo in 91% of patients, and >12 mo in 80% of patients. Conclusions This first study of changes in VC and S/MIP at 3 and 6 mo of follow-up show they are highly predictive of death in ALS.