Pseudobulbar affect as a negative prognostic indicator in amyotrophic lateral sclerosis.

Autor: Tortelli R; Unit of Neurodegenerative Diseases, Department of Clinical Research in Neurology, University of Bari 'A. Moro' at Pia Fondazione Card. G. Panico, Tricase, Lecce, Italy., Arcuti S; Unit of Biostatistics, IRCCS 'Casa Sollievo della Sofferenza', San Giovanni Rotondo, Foggia, Italy., Copetti M; Unit of Biostatistics, IRCCS 'Casa Sollievo della Sofferenza', San Giovanni Rotondo, Foggia, Italy., Barone R; Unit of Neurodegenerative Diseases, Department of Clinical Research in Neurology, University of Bari 'A. Moro' at Pia Fondazione Card. G. Panico, Tricase, Lecce, Italy., Zecca C; Unit of Neurodegenerative Diseases, Department of Clinical Research in Neurology, University of Bari 'A. Moro' at Pia Fondazione Card. G. Panico, Tricase, Lecce, Italy., Capozzo R; Unit of Neurodegenerative Diseases, Department of Clinical Research in Neurology, University of Bari 'A. Moro' at Pia Fondazione Card. G. Panico, Tricase, Lecce, Italy., Barulli MR; Unit of Neurodegenerative Diseases, Department of Clinical Research in Neurology, University of Bari 'A. Moro' at Pia Fondazione Card. G. Panico, Tricase, Lecce, Italy., Simone IL; Department of Basic Medical Sciences, Neurosciences and Sense Organs, University of Bari 'A. Moro', Bari, Italy., Logroscino G; Unit of Neurodegenerative Diseases, Department of Clinical Research in Neurology, University of Bari 'A. Moro' at Pia Fondazione Card. G. Panico, Tricase, Lecce, Italy.; Department of Basic Medical Sciences, Neurosciences and Sense Organs, University of Bari 'A. Moro', Bari, Italy.
Jazyk: angličtina
Zdroj: Acta neurologica Scandinavica [Acta Neurol Scand] 2018 Jul; Vol. 138 (1), pp. 55-61. Date of Electronic Publication: 2018 Mar 11.
DOI: 10.1111/ane.12918
Abstrakt: Objective: To evaluate whether the presence of pseudobulbar affect (PBA) in an early stage of the disease influences survival in a population-based incident cohort of amyotrophic lateral sclerosis (ALS).
Methods: Incident ALS cases, diagnosed according to El Escorial criteria, were enrolled from a prospective population-based registry in Puglia, Southern Italy. The Center for Neurologic Study-Lability Scale (CNS-LS), a self-administered questionnaire, was used to evaluate PBA. Total scores range from 7 to 35. A score ≥13 was used to identify PBA. Cox proportional hazard models were used for survival analysis. The modified C-statistic for censored survival data was used for models' discrimination. RECursive Partitioning and AMalgamation (RECPAM) analysis was used to identify subgroups of patients with different patterns of risk, depending on baseline characteristics.
Results: We enrolled 94 sporadic ALS, median age of 64 years (range: 26-80). At the censoring date, 65 of 94 (69.2%), 39 of 60 (65.0%), and 26 of 34 (76.5%) patients reached the outcome (tracheotomy/death), in the whole, non-PBA and in the PBA groups, respectively. Kaplan-Meier survival curves for the two subgroups were not significantly different (log-rank test: 1.3, P = .25). The discrimination ability of a multivariable model with demographic and clinical variables of interest was not improved by adding PBA. In the RECPAM analysis, ALSFRSr and the total score of CNS-LS scale (Conclusions: These preliminary results underlie that the presence of PBA at entry negatively influences survival in a specific subgroup of patients with ALS characterized by less functional impairment.
(© 2018 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)
Databáze: MEDLINE
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