Mandrioli J, et al. - The intent of the authors was to contemplate the role of riluzole therapy, alongside other clinical factors, on the prognosis in incident amyotrophic lateral sclerosis (ALS) cases in Emilia Romagna Region (ERR), Italy. Longer survival was reported among subjects who received riluzole for a longer period of time, independently from other prognostic factors.

Methods

• Since 2009 a registry for ALS cumulated all incident cases in ERR.
• A scrutiny was performed of the detailed clinical data from all patients diagnosed with ALS between 1.1.2009 and 31.12.2014.
• The last follow-up date was set at 31.12.2015.

Results

• A total of 681 incident cases were reported with a median tracheostomy-free survival of 40 months (95% CI 36-44) from onset and of 26 months (95% CI 24-30) from diagnosis; 573 patients (84.14%) were treated with riluzole, 207 (30.39%) patients underwent gastrostomy, 246 (36.12%) non invasive ventilation, and 103 (15.15%) invasive ventilation during the 6 years of the study.
• A median survival of 29 months was revealed among subjects who took treatment for ≥ 75% of disease duration from diagnosis vs 18 months in patients with < 75% treatment duration.
• Factors independently affecting survival included age at onset (HR 1.04, 95% CI 1.02-1.05, p < 0.001), dementia (HR 1.56, 95% CI 1.05-2.32, p=0.027), degree of diagnostic certainty (HR 0.88, 95% CI 0.78-0.98, p=0.021), gastrostomy (HR 1.46, 95% CI 1.14-1.88, p=0.003), NIV (HR 1.43, 95% CI 1.12-1.82, p=0.004), and weight loss at diagnosis (HR 1.05, 95% CI 1.03-1.07, p < 0.001), diagnostic delay (HR 0.98, 95% CI 0.97-0.99, p=0.004), and % treatment duration (HR 0.98, 95% CI 0.98-0.99, p < 0.001), as disclosed via multivariable analysis.