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Factors predicting survival in ALS: a multicenter Italian study

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Abstract

The aim of this multicenter, retrospective study is to investigate the role of clinical characteristics and therapeutic intervention on ALS prognosis. The study included patients diagnosed from January 1, 2009 to December 31, 2013 in 13 Italian referral centers for ALS located in 10 Italian regions. Caring neurologists collected a detailed phenotypic profile and follow-up data until death into an electronic database. One center collected also data from a population-based registry for ALS. 2648 incident cases were collected. The median survival time from onset to death/tracheostomy was 44 months (SE 1.18, CI 42–46). According to univariate analysis, factors related to survival from onset to death/tracheostomy were: age at onset, diagnostic delay, site of onset, phenotype, degree of certainty at diagnosis according to revised El Escorial criteria (R-EEC), presence/absence of dementia, BMI at diagnosis, patients’ provenance. In the multivariate analysis, age at onset, diagnostic delay, phenotypes but not site of onset, presence/absence of dementia, BMI, riluzole use, R-EEC criteria were independent prognostic factors of survival in ALS. We compared patients from an ALS Registry with patients from tertiary centers; the latter ones were younger, less frequently bulbar, but more frequently familial and definite at diagnosis. Our large, multicenter study demonstrated the role of some clinical and demographic factors on ALS survival, and showed some interesting differences between referral centers’ patients and the general ALS population. These results can be helpful for clinical practice, in clinical trial design and to validate new tools to predict disease progression.

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Acknowledgments

The authors thank all the collaborators of the multidisciplinary centers for motor neuron disease involved in the study: Antonio Fasano, Laura Ferri (Modena), Giovanni Novi (Genova), Rosa Capozzo (Tricase), Cinzia Femiano, Mattia Siciliano (Naples), Giulia Bisogni (Rome); Andrea Lizio, Eleonora Maestri, Claudia Tarlarini (Nemo Milano), Claudia Morelli, Federico Verde, Stefano Messina (Istituto Auxologico, Milano), Antonio Onniboni (Fondazione S. Maugeri, Mistretta), Riccardo Sideri (Fondazione S. Maugeri, Milano). Jessica Mandrioli has received research support from Regione EmiIia Romagna (Programma di ricerca Regione-Università 2010–2012, area 2, Ricerca per il Governo Clinico; Emilia Romagna Registry for Amyotrophic Lateral Sclerosis 2009–2015). Outside this work, Francesca Trojsi perceived Grants from Novartis and Maria Rosaria Monsurrò grants from Italfarmaco and Italian Association for Amyotrophic Lateral Sclerosis (AISLA). Amelia Conte and Mario Sabatelli thank I.CO.M.M. onlus, association for Amyotrophic Lateral Sclerosis research. Nicola Ticozzi received research support from the Italian Ministry of Health (Grant GR-2011-02347820—IRisALS) and from the Associazione “Io Corro con Giovanni”.

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Correspondence to Jessica Mandrioli.

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The study was performed in accordance with the ethical standards statement.

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Calvo, A., Moglia, C., Lunetta, C. et al. Factors predicting survival in ALS: a multicenter Italian study. J Neurol 264, 54–63 (2017). https://doi.org/10.1007/s00415-016-8313-y

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