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01-04-2018 | Original Communication

Riluzole and other prognostic factors in ALS: a population-based registry study in Italy

Authors: Jessica Mandrioli, Sara Angela Malerba, Ettore Beghi, Nicola Fini, Antonio Fasano, Elisabetta Zucchi, Silvia De Pasqua, Carlo Guidi, Emilio Terlizzi, Elisabetta Sette, Alessandro Ravasio, Mario Casmiro, Fabrizio Salvi, Rocco Liguori, Lucia Zinno, Yasmin Handouk, Romana Rizzi, Annamaria Borghi, Rita Rinaldi, Doriana Medici, Mario Santangelo, Enrico Granieri, Vittoria Mussuto, Marina Aiello, Salvatore Ferro, Marco Vinceti, ERRALS Group

Published in: Journal of Neurology | Issue 4/2018

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.

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Title: Riluzole and other prognostic factors in ALS: a population-based registry study in Italy
Authors: Jessica Mandrioli
Sara Angela Malerba
Ettore Beghi
Nicola Fini
Antonio Fasano
Elisabetta Zucchi
Silvia De Pasqua
Carlo Guidi
Emilio Terlizzi
Elisabetta Sette
Alessandro Ravasio
Mario Casmiro
Fabrizio Salvi
Rocco Liguori
Lucia Zinno
Yasmin Handouk
Romana Rizzi
Annamaria Borghi
Rita Rinaldi
Doriana Medici
Mario Santangelo
Enrico Granieri
Vittoria Mussuto
Marina Aiello
Salvatore Ferro
Marco Vinceti
ERRALS Group
Publication date: 01-04-2018
Publisher: Springer Berlin Heidelberg
Published in: Journal of Neurology / Issue 4/2018
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-018-8778-y

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Riluzole and other prognostic factors in ALS: a population-based registry study in Italy

Abstract

Objective

Methods

Results

Conclusions

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Objective

Methods

Results

Conclusions

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