Comorbidity of dementia with amyotrophic lateral sclerosis (ALS): insights from a large multicenter Italian cohort

Francesca Trojsi, Mattia Siciliano, Cinzia Femiano, Gabriella Santangelo, Christian Lunetta, Andrea Calvo, Cristina Moglia, Kalliopi Marinou, Nicola Ticozzi, Gianluca Drago Ferrante, Carlo Scialò, Gianni Sorarù, Amelia Conte, Yuri M. Falzone, Rosanna Tortelli, Massimo Russo, Valeria Ada Sansone, Adriano Chiò, Gabriele Mora, Barbara PolettiPaolo Volanti, Claudia Caponnetto, Giorgia Querin, Mario Sabatelli, Nilo Riva, Giancarlo Logroscino, Sonia Messina, Antonio Fasano, Maria Rosaria Monsurrò, Gioacchino Tedeschi, Jessica Mandrioli

Research output: Contribution to journalArticle

5 Citations (Scopus)

Abstract

To assess the association, at diagnosis, between amyotrophic lateral sclerosis (ALS) and dementia in a large cohort of well-characterized Italian patients. We investigated the phenotypic profile of 1638 incident patients with definite, probable or laboratory-supported probable ALS, diagnosed from January 2009 to December 2013 in 13 Italian Referral Centers, located in 10 Italian Regions, and classified in two independent subsamples accounting for presence or not of dementia. The collected ALS features, including survival and other follow-up data, were compared between the two subgroups using a one-way analysis of variance and Chi-square test, as appropriate, logistic regression models and Kaplan–Meier survival analysis. Between-subgroup comparisons showed an older age at clinical observation (p = .006), at onset and at diagnosis (p = .002) in demented versus non demented ALS patients. After adjustment for these variables, diagnosis of dementia was significantly associated with higher odds of family history of ALS (p = .001) and frontotemporal dementia (p = .003) and of bulbar onset (p = .004), and lower odds of flail leg phenotype (p = .019) and spinal onset (p = .008). The median survival time was shorter in demented versus non-demented patients, especially in case of classical, bulbar and flail limb phenotypes and both bulbar and spinal onset. Our multicenter study emphasized the importance of an early diagnosis of comorbid dementia in ALS patients, which may have clinical impact and prognostic relevance. Moreover, our results may give further inputs to validation of ALS-specific tools for the screening of cognitive impairment in clinical practice.

Original languageEnglish
Pages (from-to)1-8
Number of pages8
JournalJournal of Neurology
DOIs
Publication statusPublished - Nov 2017

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Amyotrophic Lateral Sclerosis
Comorbidity
Dementia
Logistic Models
Phenotype
Frontotemporal Dementia
Survival
Chi-Square Distribution
Survival Analysis
Multicenter Studies
Early Diagnosis
Leg
Analysis of Variance
Referral and Consultation
Extremities
Observation

Keywords

  • Amyotrophic lateral sclerosis
  • Clinical phenotype
  • Dementia
  • Survival

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

Cite this

Comorbidity of dementia with amyotrophic lateral sclerosis (ALS) : insights from a large multicenter Italian cohort. / Trojsi, Francesca; Siciliano, Mattia; Femiano, Cinzia; Santangelo, Gabriella; Lunetta, Christian; Calvo, Andrea; Moglia, Cristina; Marinou, Kalliopi; Ticozzi, Nicola; Drago Ferrante, Gianluca; Scialò, Carlo; Sorarù, Gianni; Conte, Amelia; Falzone, Yuri M.; Tortelli, Rosanna; Russo, Massimo; Sansone, Valeria Ada; Chiò, Adriano; Mora, Gabriele; Poletti, Barbara; Volanti, Paolo; Caponnetto, Claudia; Querin, Giorgia; Sabatelli, Mario; Riva, Nilo; Logroscino, Giancarlo; Messina, Sonia; Fasano, Antonio; Monsurrò, Maria Rosaria; Tedeschi, Gioacchino; Mandrioli, Jessica.

In: Journal of Neurology, 11.2017, p. 1-8.

Research output: Contribution to journalArticle

Trojsi, F, Siciliano, M, Femiano, C, Santangelo, G, Lunetta, C, Calvo, A, Moglia, C, Marinou, K, Ticozzi, N, Drago Ferrante, G, Scialò, C, Sorarù, G, Conte, A, Falzone, YM, Tortelli, R, Russo, M, Sansone, VA, Chiò, A, Mora, G, Poletti, B, Volanti, P, Caponnetto, C, Querin, G, Sabatelli, M, Riva, N, Logroscino, G, Messina, S, Fasano, A, Monsurrò, MR, Tedeschi, G & Mandrioli, J 2017, 'Comorbidity of dementia with amyotrophic lateral sclerosis (ALS): insights from a large multicenter Italian cohort', Journal of Neurology, pp. 1-8. https://doi.org/10.1007/s00415-017-8619-4
Trojsi, Francesca ; Siciliano, Mattia ; Femiano, Cinzia ; Santangelo, Gabriella ; Lunetta, Christian ; Calvo, Andrea ; Moglia, Cristina ; Marinou, Kalliopi ; Ticozzi, Nicola ; Drago Ferrante, Gianluca ; Scialò, Carlo ; Sorarù, Gianni ; Conte, Amelia ; Falzone, Yuri M. ; Tortelli, Rosanna ; Russo, Massimo ; Sansone, Valeria Ada ; Chiò, Adriano ; Mora, Gabriele ; Poletti, Barbara ; Volanti, Paolo ; Caponnetto, Claudia ; Querin, Giorgia ; Sabatelli, Mario ; Riva, Nilo ; Logroscino, Giancarlo ; Messina, Sonia ; Fasano, Antonio ; Monsurrò, Maria Rosaria ; Tedeschi, Gioacchino ; Mandrioli, Jessica. / Comorbidity of dementia with amyotrophic lateral sclerosis (ALS) : insights from a large multicenter Italian cohort. In: Journal of Neurology. 2017 ; pp. 1-8.
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AU - Trojsi, Francesca

AU - Siciliano, Mattia

AU - Femiano, Cinzia

AU - Santangelo, Gabriella

AU - Lunetta, Christian

AU - Calvo, Andrea

AU - Moglia, Cristina

AU - Marinou, Kalliopi

AU - Ticozzi, Nicola

AU - Drago Ferrante, Gianluca

AU - Scialò, Carlo

AU - Sorarù, Gianni

AU - Conte, Amelia

AU - Falzone, Yuri M.

AU - Tortelli, Rosanna

AU - Russo, Massimo

AU - Sansone, Valeria Ada

AU - Chiò, Adriano

AU - Mora, Gabriele

AU - Poletti, Barbara

AU - Volanti, Paolo

AU - Caponnetto, Claudia

AU - Querin, Giorgia

AU - Sabatelli, Mario

AU - Riva, Nilo

AU - Logroscino, Giancarlo

AU - Messina, Sonia

AU - Fasano, Antonio

AU - Monsurrò, Maria Rosaria

AU - Tedeschi, Gioacchino

AU - Mandrioli, Jessica

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N2 - To assess the association, at diagnosis, between amyotrophic lateral sclerosis (ALS) and dementia in a large cohort of well-characterized Italian patients. We investigated the phenotypic profile of 1638 incident patients with definite, probable or laboratory-supported probable ALS, diagnosed from January 2009 to December 2013 in 13 Italian Referral Centers, located in 10 Italian Regions, and classified in two independent subsamples accounting for presence or not of dementia. The collected ALS features, including survival and other follow-up data, were compared between the two subgroups using a one-way analysis of variance and Chi-square test, as appropriate, logistic regression models and Kaplan–Meier survival analysis. Between-subgroup comparisons showed an older age at clinical observation (p = .006), at onset and at diagnosis (p = .002) in demented versus non demented ALS patients. After adjustment for these variables, diagnosis of dementia was significantly associated with higher odds of family history of ALS (p = .001) and frontotemporal dementia (p = .003) and of bulbar onset (p = .004), and lower odds of flail leg phenotype (p = .019) and spinal onset (p = .008). The median survival time was shorter in demented versus non-demented patients, especially in case of classical, bulbar and flail limb phenotypes and both bulbar and spinal onset. Our multicenter study emphasized the importance of an early diagnosis of comorbid dementia in ALS patients, which may have clinical impact and prognostic relevance. Moreover, our results may give further inputs to validation of ALS-specific tools for the screening of cognitive impairment in clinical practice.

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