Acta Neurol Scand. 2018;138:55–61. wileyonlinelibrary.com/journal/ane
© 2018 John Wiley & Sons A/S.
Published by John Wiley & Sons Ltd
1 | BACKGROUND
Pseudobulbar affect (PBA) is a frequent symptom in amyotrophic
lateral sclerosis (ALS), being present in up to 50% of cases at any
time point of the disease
and in almost one in four cases at diagno-
It is described as the presence of involuntary and uncontrollable
outbursts of laughing and/or crying incongruous or disproportionate
to the patient’s emotional state.
Pseudobulbar affect may impair patients’ quality of life but is
often under- recognized and undertreated.
In a previous study, PBA has been related to a more severe ALS
phenotype at diagnosis.
To date, there are no studies that evaluate
the role of PBA as prognostic indicator in ALS.
The aim of this study was to evaluate whether the presence
of PBA in an early stage of the disease can predict survival in a
population- based incident cohort of ALS.
Accepted: 19 February 2018
Pseudobulbar affect as a negative prognostic indicator in
amyotrophic lateral sclerosis
| S. Arcuti
| M. Copetti
| R. Barone
| C. Zecca
| R. Capozzo
M. R. Barulli
| I. L. Simone
| G. Logroscino
Unit of Neurodegenerative Diseases,
Department of Clinical Research in
Neurology, University of Bari “A. Moro” at
Pia Fondazione Card. G. Panico, Tricase,
Unit of Biostatistics, IRCCS “Casa Sollievo
della Sofferenza”, San Giovanni Rotondo,
Department of Basic Medical Sciences,
Neurosciences and Sense Organs, University
of Bari “A. Moro”, Bari, Italy
Giancarlo Logroscino, Department of Basic
Medical Sciences, Neurosciences, and Sense
Organs, University of Bari “Aldo Moro,” Bari,
Italy and Department of Clinical Research
in Neurology, University of Bari Aldo Moro,
“Pia Fondazione Cardinale G. Panico,”
Tricase, Lecce, Italy.
European Community’s Seventh Framework
Programme FP7/2007- 2013 under grant
agreement 259867. FP7 Health259867.
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 en-
rolled from a prospective population- based registry in Puglia, Southern Italy. The
Center for Neurologic Study- Lability Scale (CNS- LS), a self- administered question-
used to identify PBA. Cox proportional hazard models were used for survival analy-
sis. The modified C- statistic for censored survival data was used for models’ discrimi-
nation. RECursive Partitioning and AMalgamation (RECPAM) analysis was used to
identify subgroups of patients with different patterns of risk, depending on baseline
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 mul-
tivariable 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 character-
ized by less functional impairment.
amyotrophic lateral sclerosis, pseudobulbar affect, survival