Factors predicting survival in ALS: a multicenter Italian study

Andrea Calvo, Cristina Moglia, Christian Lunetta, Kalliopi Marinou, Nicola Ticozzi, Gianluca Drago Ferrante, Carlo Scialo, Gianni Sorarù, Francesca Trojsi, Amelia Conte, Yuri M. Falzone, Rosanna Tortelli, Massimo Russo, Adriano Chiò, Valeria Ada Sansone, Gabriele Mora, Vincenzo Silani, Paolo Volanti, Claudia Caponnetto, Giorgia QuerinMaria Rosaria Monsurrò, Mario Sabatelli, Nilo Riva, Giancarlo Logroscino, Sonia Messina, Nicola Fini, Jessica Mandrioli

Research output: Contribution to journalArticle

32 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.

Original languageEnglish
Pages (from-to)54-63
Number of pages10
JournalJournal of Neurology
DOIs
Publication statusPublished - Jan 2017

Fingerprint

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

Keywords

  • ALS
  • Population-based registries
  • Prognostic factors
  • Referral centers
  • Survival

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

Cite this

Factors predicting survival in ALS : a multicenter Italian study. / Calvo, Andrea; Moglia, Cristina; Lunetta, Christian; Marinou, Kalliopi; Ticozzi, Nicola; Ferrante, Gianluca Drago; Scialo, Carlo; Sorarù, Gianni; Trojsi, Francesca; Conte, Amelia; Falzone, Yuri M.; Tortelli, Rosanna; Russo, Massimo; Chiò, Adriano; Sansone, Valeria Ada; Mora, Gabriele; Silani, Vincenzo; Volanti, Paolo; Caponnetto, Claudia; Querin, Giorgia; Monsurrò, Maria Rosaria; Sabatelli, Mario; Riva, Nilo; Logroscino, Giancarlo; Messina, Sonia; Fini, Nicola; Mandrioli, Jessica.

In: Journal of Neurology, 01.2017, 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, pp. 54-63. https://doi.org/10.1007/s00415-016-8313-y
Calvo, Andrea ; Moglia, Cristina ; Lunetta, Christian ; Marinou, Kalliopi ; Ticozzi, Nicola ; Ferrante, Gianluca Drago ; Scialo, Carlo ; Sorarù, Gianni ; Trojsi, Francesca ; Conte, Amelia ; Falzone, Yuri M. ; Tortelli, Rosanna ; Russo, Massimo ; Chiò, Adriano ; Sansone, Valeria Ada ; Mora, Gabriele ; Silani, Vincenzo ; Volanti, Paolo ; Caponnetto, Claudia ; Querin, Giorgia ; Monsurrò, Maria Rosaria ; Sabatelli, Mario ; Riva, Nilo ; Logroscino, Giancarlo ; Messina, Sonia ; Fini, Nicola ; Mandrioli, Jessica. / Factors predicting survival in ALS : a multicenter Italian study. In: Journal of Neurology. 2017 ; pp. 54-63.
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AU - Calvo, Andrea

AU - Moglia, Cristina

AU - Lunetta, Christian

AU - Marinou, Kalliopi

AU - Ticozzi, Nicola

AU - Ferrante, Gianluca Drago

AU - Scialo, Carlo

AU - Sorarù, Gianni

AU - Trojsi, Francesca

AU - Conte, Amelia

AU - Falzone, Yuri M.

AU - Tortelli, Rosanna

AU - Russo, Massimo

AU - Chiò, Adriano

AU - Sansone, Valeria Ada

AU - Mora, Gabriele

AU - Silani, Vincenzo

AU - Volanti, Paolo

AU - Caponnetto, Claudia

AU - Querin, Giorgia

AU - Monsurrò, Maria Rosaria

AU - Sabatelli, Mario

AU - Riva, Nilo

AU - Logroscino, Giancarlo

AU - Messina, Sonia

AU - Fini, Nicola

AU - Mandrioli, Jessica

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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.

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