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AIM: The aim of the study was to assess the consequences of early and systematic nutritional intervention on the clinical conditions of amyotrophic lateral sclerosis (ALS) patients and on the opportunity to maintain a good nutritional status for as long as possible.; METHODS: Thirty-three subjects with ALS. Protocol Group: 12 subjects (9 M and 3 F) monitored according to a precise nutritional intervention protocol. Control Group: 21 subjects (10 M and 11 F) monitored before applying the protocol.; RESULTS: Data recorded at the time of initial assessment were compared and expressed as the mean standard deviation for the Protocol Group vs. the Control Group: BMI (kg/m2) 23.64.1 vs. 21.63.5; weight loss as a percentage of usual weight 6.67.9 vs. 16.38.8 (P=0.003). At six months: weight loss as a percentage of usual weight 4.96.2 vs. 16.910.2 (P=0.002). At 12 months: weight loss as a percentage of usual weight 7.37.1 vs. 17.511.1 (P=0.03). At the first follow-up visit, fewer patients in the Protocol Group were receiving enteral nutrition (25%) than patients in the Control Group (60%). At six-month follow-up visit: 30% vs. 68%. Standard enteral nutrition formulas were used. One year after initial assessment, the mortality rate was 17% for the Protocol Group, whereas it was 24% at six months and 33% after one year for the Control Group.; CONCLUSION: If patients are treated before any significant weight loss occurs, early and specific nutritional intervention allows good nutritional status to be maintained for a longer period; if artificial nutrition is required, standard diets are able to ensure adequate clinical results.