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Background: Malnutrition is a prognostic factor in Amyotrophic Lateral Sclerosis (ALS). Sometimes, this condition is underdiagnosed, and it might influence on disease progression., Aims: To evaluate a) nutritional status at the beginning of specialized nutritional treatment and b) the influence of initial nutritional status on disease evolution and survival in a group of patients with amyotrophic lateral sclerosis (ALS)., Methods: An interhospital registry of patients with motor neuron disease treated at the Clinical Nutrition Clinics of six hospitals in the region of Castilla y León in Spain was created. The study was developed from January 2015 to December 2017. An anamnesis, affiliation data, past medical history, disease evolution, nutritional history and an anthropometry and bioelectrical impedance analysis were performed at baseline. The mortality rate was compared among those patients with worse nutritional status at the beginning of the follow-up against those with a better nutritional situation using two tools: The Subjective Global Assessment (SGA) and the criteria of the Global Leadership Initiative for Malnutrition (GLIM)., Results: A total of 93 patients were analysed. The median age of the patients was 67 (57.5-75.5) years. The median Body Mass Index was 24.4 (21.7-25.9) kg/m 2 and the median percentage of weight loss was 9.32 (2.7-17.6)% without differences between the onset type. According to the SGA, 27 (29%) patients were in grade A; 43 (46.3%) patients were in grade B and 23 (24.7%) were in grade C. According to the new GLIM malnutrition criteria, 45 patients (48.4%) had malnutrition. Patients with worse nutritional status had a lower survival median with both SGA (SGA A: 20.5 (10.2-35) months vs SGA B-C: 12 (5.2-23.7) months (p = 0.03)) or the new GLIM criteria according to severity (severe malnutrition: 18 (5-24) months vs. no severe malnutrition: 20 (12-33) months (p = 0.01)). In the multivariate analysis, malnutrition measured by SGA was an independent risk factor (HR: 4.6 (1.5-13.9) p = 0.007) for survival over 15 months when adjusted for age, sex and type of onset of ALS., Conclusions: Patients with ALS have a severe deterioration in nutritional status when analysed using a classical malnutrition test (SGA) or a new one (GLIM criteria). Patients with a better nutritional situation according to SGA and GLIM severity classification were associated with a longer survival time., Competing Interests: Conflicts of interest There was no conflict of interest of the authors about the development of the study., (Copyright © 2020 Elsevier Ltd and European Society for Clinical Nutrition and Metabolism. All rights reserved.)