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Heterogeneity in ALSFRS-R decline and survival: a population-based study in Italy

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Abstract

Very few studies examined trend over time of the revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) and factors influencing it; previous studies, then, included only patients attending tertiary ALS Centres. We studied ALSFRS-R decline, factors influencing this trend and survival in a population-based setting. From 2009 onwards, a prospective registry records all incident ALS cases among residents in Emilia Romagna (population: 4.4 million). For each patient, demographic and clinical details (including ALSFRS-R) are collected by caring physicians at each follow-up. Analysis was performed on 402 incident cases (1279 ALSFRS-R assessments). The average decline of the ALSFRS-R was 0.60 points/month during the first year after diagnosis and 0.34 points/month in the second year. ALSFRS-R decline was heterogeneous among subgroups. Repeated measures mixed model showed that ALSFRS-R score decline was influenced by age at onset (p < 0.01), phenotype (p = 0.01), body mass index (BMI) (p < 0.01), progression rate at diagnosis (ΔFS) (p < 0.01), El Escorial Criteria—Revised (p < 0.01), and FVC% at diagnosis (p < 0.01). Among these factors, at multivariate analysis, only age, site of onset and ΔFS independently influenced survival. In this first population-based study on ALSFRS-R trend, we confirm that ALSFRS-R decline is not homogeneous among ALS patients and during the disease. Factors influencing ALSFRS-R decline may not match with those affecting survival. These disease modifiers should be taken into consideration for trials design and in clinical practice during discussions with patients on prognosis.

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Acknowledgments

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 ALS is supported by a grant from the Emilia Romagna Regional Health Authority. The authors acknowledge all the ERRALS groups.

Author information

Authors and Affiliations

  1. Department of Neuroscience, St. Agostino-Estense Hospital, Via Pietro Giardini n. 1355, 41100, Modena, Italy

    Jessica Mandrioli, Nicola Fini & Eleni Georgoulopoulou

  2. Department of Neurology, Bufalini Hospital, Cesena, Italy

    Sara Biguzzi

  3. Department of Neurology, Forlì Hospital, Forlì, Italy

    Carlo Guidi

  4. Department of Neurology, St. Anna Hospital, Ferrara, Italy

    Elisabetta Sette

  5. Department of Neurology, G. Da Saliceto Hospital, Piacenza, Italy

    Emilio Terlizzi

  6. Department of Neurology, Infermi Hospital, Rimini, Italy

    Alessandro Ravasio

  7. Department of Neurology, Faenza and Ravenna Hospital, Ravenna, Italy

    Mario Casmiro

  8. Neurology Unit, Bellaria Hospital, IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy

    Fabrizio Salvi

  9. Department of Biomedical and Neuromotor Sciences, University of Bologna and IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy

    Rocco Liguori & Silvia De Pasqua

  10. Department of Neurology, IRCCS Arcispedale Santa Maria Nuova, Reggio Emilia, Italy

    Romana Rizzi

  11. Department of Neuroscience, Neurology Unit, University of Parma, Parma, Italy

    Vladimiro Pietrini

  12. Neurology Unit, Maggiore Hospital, IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy

    Annamaria Borghi

  13. Department of Neuroscience, St. Orsola-Malpighi University Hospital, Bologna, Italy

    Rita Rinaldi

  14. Department of Neurology, Fidenza Hospital, Parma, Italy

    Elisabetta Chierici

  15. Department of Neurology, Carpi Hospital, Modena, Italy

    Mario Santangelo

  16. Department of Neuroscience, University of Ferrara, Ferrara, Italy

    Enrico Granieri

  17. Department of Neurology, Imola Hospital, Bologna, Italy

    Vittoria Mussuto

  18. Department of Neuroscience, University of Modena and Reggio Emilia, Modena, Italy

    Antonio Fasano

  19. Department of Hospital Services, Emilia Romagna Regional Health Authority, Bologna, Italy

    Salvatore Ferro

  20. Neuroepidemiology Unit, IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy

    Roberto D’Alessandro

Authors
  1. Jessica Mandrioli
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  2. Sara Biguzzi
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  3. Carlo Guidi
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  4. Elisabetta Sette
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  5. Emilio Terlizzi
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  6. Alessandro Ravasio
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  7. Mario Casmiro
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  8. Fabrizio Salvi
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  9. Rocco Liguori
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  10. Romana Rizzi
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  11. Vladimiro Pietrini
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  12. Annamaria Borghi
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  13. Rita Rinaldi
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  14. Nicola Fini
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  15. Elisabetta Chierici
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  16. Mario Santangelo
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  17. Enrico Granieri
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  18. Vittoria Mussuto
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  19. Silvia De Pasqua
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  20. Eleni Georgoulopoulou
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  21. Antonio Fasano
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  22. Salvatore Ferro
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  23. Roberto D’Alessandro
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Consortia

ERRALS Group

Corresponding author

Correspondence to Jessica Mandrioli.

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The authors declare no conflicts of interest.

Appendix: ERRALS group

Appendix: ERRALS group

Project coordinator: J. Mandrioli

Collaborating centres:

Department of Neuroscience, St. Agostino Estense Hospital, Modena, Italy (J. Mandrioli, N. Fini, E. Georgoulopoulou, A. Fasano, P. Nichelli)

Department of Neurology, Bufalini Hospital, Cesena, Italy (S. Biguzzi, E. Venturini, M. G. Passarin)

Department of Neurology, Forlì Hospital, Forlì, Italy (C. Guidi, W. Neri)

Department of Neurology, St. Anna Hospital, Ferrara, Italy (E. Sette, V. Tugnoli, 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, N. Marcello)

Department of Neuroscience, University of Parma, Parma, Italy (A. Grassi, L. Delay, V. Pietrini)

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 (E. Chierici, E. Montanari)

Department of Neurology, Carpi Hospital, Modena, Italy (M. Santangelo, S. Amidei, G. Greco)

Department of Neuroscience, University of Ferrara, Ferrara, Italy (I. Casetta, E. Groppo, 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, T. Sacquegna)

Department of Neuroscience, St. Orsola-Malpighi University Hospital, 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)

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Mandrioli, J., Biguzzi, S., Guidi, C. et al. Heterogeneity in ALSFRS-R decline and survival: a population-based study in Italy. Neurol Sci 36, 2243–2252 (2015). https://doi.org/10.1007/s10072-015-2343-6

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  • DOI: https://doi.org/10.1007/s10072-015-2343-6

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