Supplementary Material for: Population-Based Evidence that Survival in Amyotrophic Lateral Sclerosis Is Related to Weight Loss at Diagnosis

<p><b><i>Background/Aims:</i></b> In a population-based setting, we aimed to (i) describe weight loss (WL) of amyotrophic lateral sclerosis (ALS) patients at the time of diagnosis and (ii) evaluate the association between WL and survival. <b><i>Methods:</i></b> All patients recruited in the FRALim register (2000-2013) were considered to be included in this study. Time-to-death analyses were performed using a multivariable Cox model. Model discrimination and calibration were assessed. <b><i>Results:</i></b> Among 322 patients in the register, 261 (81%) were included. At the time of diagnosis, 50.6% of patients reported a WL of more than 5%: 14.6% with WL between 5 and 10% and 36.0% with a WL of more than 10%. WL was independently associated with survival (p = 0.002). Patients with a WL of 10% or more experienced a 45% increase in the risk of death (95% CI 6-99) with respect to patients with a WL lower than 5% or no WL. The introduction of WL significantly improved the model's discrimination achieving a survival C statistic of 79.5% (95% CI 75.6-83.5, p = 0.006) at 12 months. <b><i>Conclusion:</i></b> More than 50% of ALS patients experience a WL of more than 5% at the time of diagnosis. This finding highlights the need for randomized trials to evaluate the effect of nutritional interventions to improve ALS survival.