Pseudobulbar affect as a negative prognostic indicator in amyotrophic lateral sclerosis

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. REC ursive Partitioning and AM algamation ( 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, ALSFRS r and the total score of CNS ‐ LS scale (

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