Factors predicting survival in ALS: a multicenter Italian study

Andrea Calvo, Cristina Moglia, C Lunetta, K Marinou, N Ticozzi, GD Ferrante, C Scialo, G Sorarù, Francesca Trojsi, A Conte, YM Falzone, R Tortelli, M Russo, A Chiò, VA Sansone, G Mora, V Silani, P Volanti, C Caponnetto, G Querin & 7 others MR Monsurrò, Mario Sabatelli, N Riva, Giancarlo Logroscino, S Messina, Nicola Fini, Jessica Mandrioli

Research output: Contribution to journalArticle

29 Citations (Scopus)

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. © 2016 Springer-Verlag Berlin Heidelberg
Original languageEnglish
Pages (from-to)54-63
Number of pages10
JournalJournal of Neurology
Volume264
Issue number1
DOIs
Publication statusPublished - 2017

Fingerprint

Multicenter Studies
Survival
Tracheostomy
Age of Onset
Dementia
Registries
Referral and Consultation
Riluzole
Phenotype
Berlin
Population
Statistical Factor Analysis
Disease Progression
Multivariate Analysis
Retrospective Studies
Demography
Clinical Trials
Databases
Therapeutics

Cite this

Calvo, A., Moglia, C., Lunetta, C., Marinou, K., Ticozzi, N., Ferrante, GD., ... Mandrioli, J. (2017). Factors predicting survival in ALS: a multicenter Italian study. Journal of Neurology, 264(1), 54-63. https://doi.org/10.1007/s00415-016-8313-y

Factors predicting survival in ALS: a multicenter Italian study. / Calvo, Andrea; Moglia, Cristina; Lunetta, C; Marinou, K; Ticozzi, N; Ferrante, GD; Scialo, C; Sorarù, G; Trojsi, Francesca; Conte, A; Falzone, YM; Tortelli, R; Russo, M; Chiò, A; Sansone, VA; Mora, G; Silani, V; Volanti, P; Caponnetto, C; Querin, G; Monsurrò, MR; Sabatelli, Mario; Riva, N; Logroscino, Giancarlo; Messina, S; Fini, Nicola; Mandrioli, Jessica.

In: Journal of Neurology, Vol. 264, No. 1, 2017, p. 54-63.

Research output: Contribution to journalArticle

Calvo, A, Moglia, C, Lunetta, C, Marinou, K, Ticozzi, N, Ferrante, GD, Scialo, C, Sorarù, G, Trojsi, F, Conte, A, Falzone, YM, Tortelli, R, Russo, M, Chiò, A, Sansone, VA, Mora, G, Silani, V, Volanti, P, Caponnetto, C, Querin, G, Monsurrò, MR, Sabatelli, M, Riva, N, Logroscino, G, Messina, S, Fini, N & Mandrioli, J 2017, 'Factors predicting survival in ALS: a multicenter Italian study', Journal of Neurology, vol. 264, no. 1, pp. 54-63. https://doi.org/10.1007/s00415-016-8313-y
Calvo, Andrea ; Moglia, Cristina ; Lunetta, C ; Marinou, K ; Ticozzi, N ; Ferrante, GD ; Scialo, C ; Sorarù, G ; Trojsi, Francesca ; Conte, A ; Falzone, YM ; Tortelli, R ; Russo, M ; Chiò, A ; Sansone, VA ; Mora, G ; Silani, V ; Volanti, P ; Caponnetto, C ; Querin, G ; Monsurrò, MR ; Sabatelli, Mario ; Riva, N ; Logroscino, Giancarlo ; Messina, S ; Fini, Nicola ; Mandrioli, Jessica. / Factors predicting survival in ALS: a multicenter Italian study. In: Journal of Neurology. 2017 ; Vol. 264, No. 1. pp. 54-63.
@article{519b2ea7bd924af9990e4db7349a4602,
title = "Factors predicting survival in ALS: a multicenter Italian study",
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. {\circledC} 2016 Springer-Verlag Berlin Heidelberg",
author = "Andrea Calvo and Cristina Moglia and C Lunetta and K Marinou and N Ticozzi and GD Ferrante and C Scialo and G Sorar{\`u} and Francesca Trojsi and A Conte and YM Falzone and R Tortelli and M Russo and A Chi{\`o} and VA Sansone and G Mora and V Silani and P Volanti and C Caponnetto and G Querin and MR Monsurr{\`o} and Mario Sabatelli and N Riva and Giancarlo Logroscino and S Messina and Nicola Fini and Jessica Mandrioli",
year = "2017",
doi = "10.1007/s00415-016-8313-y",
language = "English",
volume = "264",
pages = "54--63",
journal = "Journal of Neurology",
issn = "0340-5354",
publisher = "Dr. Dietrich Steinkopff Verlag GmbH and Co. KG",
number = "1",

}

TY - JOUR

T1 - Factors predicting survival in ALS: a multicenter Italian study

AU - Calvo, Andrea

AU - Moglia, Cristina

AU - Lunetta, C

AU - Marinou, K

AU - Ticozzi, N

AU - Ferrante, GD

AU - Scialo, C

AU - Sorarù, G

AU - Trojsi, Francesca

AU - Conte, A

AU - Falzone, YM

AU - Tortelli, R

AU - Russo, M

AU - Chiò, A

AU - Sansone, VA

AU - Mora, G

AU - Silani, V

AU - Volanti, P

AU - Caponnetto, C

AU - Querin, G

AU - Monsurrò, MR

AU - Sabatelli, Mario

AU - Riva, N

AU - Logroscino, Giancarlo

AU - Messina, S

AU - Fini, Nicola

AU - Mandrioli, Jessica

PY - 2017

Y1 - 2017

N2 - 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. © 2016 Springer-Verlag Berlin Heidelberg

AB - 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. © 2016 Springer-Verlag Berlin Heidelberg

U2 - 10.1007/s00415-016-8313-y

DO - 10.1007/s00415-016-8313-y

M3 - Article

VL - 264

SP - 54

EP - 63

JO - Journal of Neurology

JF - Journal of Neurology

SN - 0340-5354

IS - 1

ER -