Jessica Mandrioli1, Sara Angela Malerba2, Ettore Beghi3, Nicola Fini2, Antonio Fasano2, Elisabetta Zucchi2, Silvia De Pasqua4, Carlo Guidi5, Emilio Terlizzi6, Elisabetta Sette7, Alessandro Ravasio8, Mario Casmiro9, Fabrizio Salvi10, Rocco Liguori4, Lucia Zinno11, Yasmin Handouk12, Romana Rizzi13, Annamaria Borghi14, Rita Rinaldi15, Doriana Medici16, Mario Santangelo17, Enrico Granieri18, Vittoria Mussuto19, Marina Aiello20, Salvatore Ferro21, Marco Vinceti22. 1. Department of Neuroscience, St. Agostino-Estense Hospital, Azienda Ospedaliero Universitaria di Modena, University of Modena and Reggio Emilia, Via Pietro Giardini n. 1355, 41100, Modena, Italy. j.mandrioli@ausl.mo.it. 2. Department of Neuroscience, St. Agostino-Estense Hospital, Azienda Ospedaliero Universitaria di Modena, University of Modena and Reggio Emilia, Via Pietro Giardini n. 1355, 41100, Modena, Italy. 3. Department of Neuroscience, IRCCS-Istituto di Ricerche Farmacologiche Mario Negri, Milan, Italy. 4. Department of Biomedical and Neuromotor Sciences, University of Bologna and IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy. 5. Department of Neurology, Forlì Hospital, Forlì, Italy. 6. Department of Neurology, G. Da Saliceto Hospital, Piacenza, Italy. 7. Department of Neurology, St. Anna Hospital, Ferrara, Italy. 8. Department of Neurology, Infermi Hospital, Rimini, Italy. 9. Department of Neurology, Faenza and Ravenna Hospital, Ravenna, Italy. 10. Neurology Unit, Bellaria Hospital, IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy. 11. Department of Neuroscience, Neurology Unit, University of Parma, Parma, Italy. 12. Department of Neurology, Bufalini Hospital, Cesena, Italy. 13. Department of Neurology, IRCCS Arcispedale Santa Maria Nuova, Reggio Emilia, Italy. 14. Neurology Unit, Maggiore Hospital, IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy. 15. Department of Neuroscience, St. Orsola-Malpighi University Hospital, Bologna, Italy. 16. Department of Neurology, Fidenza Hospital, Parma, Italy. 17. Department of Neurology, Carpi Hospital, Modena, Italy. 18. Department of Neuroscience, University of Ferrara, Ferrara, Italy. 19. Department of Neurology, Imola Hospital, Bologna, Italy. 20. Department of Clinical and Experimental Medicine, Respiratory Disease and Lung Function Unit, University of Parma, Parma, Italy. 21. Department of Hospital Services, Emilia Romagna Regional Health Authority, Bologna, Italy. 22. Environmental, Genetic and Nutritional Epidemiology Research Center (CREAGEN), University of Modena and Reggio Emilia Medical School, Modena, Italy.
Abstract
OBJECTIVE: In this prospective population-based registry study on ALS survival, we investigated the role of riluzole treatment, together with other clinical factors, on the prognosis in incident ALS cases in Emilia Romagna Region (ERR), Italy. METHODS: A registry for ALS has been collecting all incident cases in ERR since 2009. Detailed clinical data from all patients diagnosed with ALS between 1.1.2009 and 31.12.2014 have been analyzed for this study, with last follow up date set at 31.12.2015. RESULTS: During the 6 years of the study, there were 681 incident cases 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. Patients who took treatment for ≥ 75% of disease duration from diagnosis had a median survival of 29 months compared to 18 months in patients with < 75% treatment duration. In multivariable analysis, factors independently influencing survival were 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). CONCLUSIONS: Independently from other prognostic factors, patients who received riluzole for a longer period of time survived longer, but further population based studies are needed to verify if long-tem use of riluzole prolongs survival.
OBJECTIVE: In this prospective population-based registry study on ALS survival, we investigated the role of riluzole treatment, together with other clinical factors, on the prognosis in incident ALS cases in Emilia Romagna Region (ERR), Italy. METHODS: A registry for ALS has been collecting all incident cases in ERR since 2009. Detailed clinical data from all patients diagnosed with ALS between 1.1.2009 and 31.12.2014 have been analyzed for this study, with last follow up date set at 31.12.2015. RESULTS: During the 6 years of the study, there were 681 incident cases 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. Patients who took treatment for ≥ 75% of disease duration from diagnosis had a median survival of 29 months compared to 18 months in patients with < 75% treatment duration. In multivariable analysis, factors independently influencing survival were 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). CONCLUSIONS: Independently from other prognostic factors, patients who received riluzole for a longer period of time survived longer, but further population based studies are needed to verify if long-tem use of riluzole prolongs survival.
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