Journal of Neurology

, Volume 265, Issue 4, pp 817–827 | Cite as

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

  • 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
Original Communication



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.


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.


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).


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.


Amyotrophic lateral sclerosis Survival Prognostic factors Therapeutic intervention Riluzole 



The authors acknowledge ERRALS group: Project coordinator: J. Mandrioli. Collaborating centers: Department of Neuroscience, St. Agostino Estense Hospital, Azienda Ospedaliero Universitaria di Modena, Italy (J. Mandrioli, N. Fini, A. Fasano, E. Zucchi, A. Gessani, L. Ferri, S. Malerba, P. Nichelli). Department of Neurology, Bufalini Hospital, Cesena, Italy (Y. Handouk, S. Biguzzi, W. Neri). Department of Neurology, Forlì Hospital, Forlì, Italy (C. Guidi, W. Neri). Department of Neurosciences and Rehabilitation, St Anna Hospital, Ferrara (E. Sette, V. Tugnoli and M. R. Tola). Department of Neurology, G. Da Saliceto Hospital, Piacenza, Italy (E. Terlizzi, D. Guidetti). Department of Neurology, Infermi Hospital, Rimini, Italy (M. Currò Dossi, M. Pasquinelli, J. Andruccioli, A. Ravasio). Department of Neurology, Faenza and Ravenna Hospital, Ravenna, Italy (M. Casmiro,F. Rasi). Department of Neurology and IRCCS Istituto delle Scienze Neurologiche di Bologna, Bellaria Hospital, Bologna, Italy (F. Salvi, I. Bartolomei, R. Michelucci). Department of Biomedical and Neuromotor Science and IRCCS Istituto delle Scienze Neurologiche di Bologna, University of Bologna, Bologna, Italy (P. Avoni, S. De Pasqua, R. Liguori). Department of Neurology, IRCCS Arcispedale Santa Maria Nuova, Reggio Emilia, Italy (R. Rizzi, E. Canali, L. Codeluppi, F. Valzania). Department of Neuroscience, University of Parma, Parma, Italy (A. Taga, V. Pietrini, M. C. Trapasso, and L. Zinno). Department of Clinical and Experimental Medicine, Respiratory Disease and Lung Function Unit, University of Parma, Parma, Italy (M. Aiello). Department of Neurology, Fidenza Hospital, Parma, Italy (D. Medici, E. Chierici, E. Montanari). Department of Neurology, Carpi Hospital, Modena, Italy (M. Santangelo, S. Amidei). Department of Biomedical and Specialty Surgical Sciences, University of Ferrara, Ferrara, Italy (I. Casetta, M. Pugliatti, E. Della Coletta, V. Govoni, E. Granieri). Department of Neurology, Imola Hospital, Bologna, Italy (P. De Massis, V. Mussuto). Department of Neurology and IRCCS Istituto delle Scienze Neurologiche di Bologna, Maggiore Hospital, Bologna, Italy (A. Borghi, A. Gabellini, R. Michelucci). Neurology Unit, St. Orsola-Malpighi University Hospital, AOU di Bologna, Italy (R. Rinaldi, F. Cirignotta). Department of Hospital Services, Emilia Romagna Regional Health Authority, Bologna, Italy (S. Ferro). Neuroepidemiology Unit, IRCCS Istituto delle Scienze Neurologiche di Bologna, Italy (R. D’Alessandro). CREAGEN Research Center for Environmental, Genetic and Nutritional Epidemiology, University of Modena and Reggio Emilia, Modena, Italy.


Emilia Romagna Registry for ALS is supported by a Grant from the Emilia Romagna Regional Health Authority.

Compliance with ethical standards

Conflicts of interest

The authors declare no conflicts of interest.

Ethical standards

The study has been approved by the ethics committees of ERR and has therefore been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki.


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  • Jessica Mandrioli
    • 1
  • Sara Angela Malerba
    • 1
  • Ettore Beghi
    • 2
  • Nicola Fini
    • 1
  • Antonio Fasano
    • 1
  • Elisabetta Zucchi
    • 1
  • Silvia De Pasqua
    • 3
  • Carlo Guidi
    • 4
  • Emilio Terlizzi
    • 5
  • Elisabetta Sette
    • 6
  • Alessandro Ravasio
    • 7
  • Mario Casmiro
    • 8
  • Fabrizio Salvi
    • 9
  • Rocco Liguori
    • 3
  • Lucia Zinno
    • 10
  • Yasmin Handouk
    • 11
  • Romana Rizzi
    • 12
  • Annamaria Borghi
    • 13
  • Rita Rinaldi
    • 14
  • Doriana Medici
    • 15
  • Mario Santangelo
    • 16
  • Enrico Granieri
    • 17
  • Vittoria Mussuto
    • 18
  • Marina Aiello
    • 19
  • Salvatore Ferro
    • 20
  • Marco Vinceti
    • 21
  • ERRALS Group
  1. 1.Department of Neuroscience, St. Agostino-Estense Hospital, Azienda Ospedaliero Universitaria di ModenaUniversity of Modena and Reggio EmiliaModenaItaly
  2. 2.Department of NeuroscienceIRCCS-Istituto di Ricerche Farmacologiche Mario NegriMilanItaly
  3. 3.Department of Biomedical and Neuromotor SciencesUniversity of Bologna and IRCCS Istituto delle Scienze Neurologiche di BolognaBolognaItaly
  4. 4.Department of NeurologyForlì HospitalForlìItaly
  5. 5.Department of NeurologyG. Da Saliceto HospitalPiacenzaItaly
  6. 6.Department of NeurologySt. Anna HospitalFerraraItaly
  7. 7.Department of NeurologyInfermi HospitalRiminiItaly
  8. 8.Department of NeurologyFaenza and Ravenna HospitalRavennaItaly
  9. 9.Neurology UnitBellaria Hospital, IRCCS Istituto delle Scienze Neurologiche di BolognaBolognaItaly
  10. 10.Department of Neuroscience, Neurology UnitUniversity of ParmaParmaItaly
  11. 11.Department of NeurologyBufalini HospitalCesenaItaly
  12. 12.Department of NeurologyIRCCS Arcispedale Santa Maria NuovaReggio EmiliaItaly
  13. 13.Neurology Unit, Maggiore HospitalIRCCS Istituto delle Scienze Neurologiche di BolognaBolognaItaly
  14. 14.Department of NeuroscienceSt. Orsola-Malpighi University HospitalBolognaItaly
  15. 15.Department of NeurologyFidenza HospitalParmaItaly
  16. 16.Department of NeurologyCarpi HospitalModenaItaly
  17. 17.Department of NeuroscienceUniversity of FerraraFerraraItaly
  18. 18.Department of NeurologyImola HospitalBolognaItaly
  19. 19.Department of Clinical and Experimental Medicine, Respiratory Disease and Lung Function UnitUniversity of ParmaParmaItaly
  20. 20.Department of Hospital Services, Emilia Romagna Regional Health AuthorityBolognaItaly
  21. 21.Environmental, Genetic and Nutritional Epidemiology Research Center (CREAGEN)University of Modena and Reggio Emilia Medical SchoolModenaItaly

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