title: Pseudobulbar affect (PBA) in an incident ALS cohort: results from the Apulia registry (SLAP).
abstract: The aim of this study is to investigate the frequency and the clinical correlations of pseudobulbar affect (PBA) in a population-based incident cohort of ALS patients. Incident ALS cases, diagnosed in 2011 and 2012, according to El Escorial criteria were enrolled from a prospective population-based registry in Apulia, Southern Italy. Neurological status was assessed using a standard neurological examination and the revised ALS Functional Rating Scale (ALSFRSr). The Center for Neurologic Study-Lability Scale (CNS-LS), a self-administered questionnaire, was used to evaluate the presence and severity of PBA. Total scores range from 7 to 35. A score ≥13 was used to identify the presence of PBA. One-hundred thirty-two sporadic incident ALS cases were enrolled. Median disease duration was 20 months (range 2-143), median onset-diagnosis interval (ODI) 12 months (range 2-131), median ALSFRSr at baseline 36/48 (range 2-47) and median ALSFRSr bulbar sub-score 10/12 (range 0-12). Neurological examination revealed presence of PBA in 34/132 patients (26%). Pathological CNS-LS score was found in 45/132 patients (34%). Median total CNS-LS score was 9/35 (range 7-29). The subgroup with pathological CNS-LS was characterized by a short disease duration from symptom onset, ODI, time to diffusion to a second region, time to generalization and ALSFRSr bulbar sub-score, bulbar onset, "definite" diagnostic category, bulbar upper motor-neuron involvement and presence of PBA at neurological examination. In population-based setting, one-third of ALS patients present PBA at diagnosis. The presence of PBA is associated with bulbar UMN involvement and markers of a more severe phenotype.
authors.affiliation: Unit of Neurodegenerative Diseases, Department of Clinical Research in Neurology, University of Bari "Aldo Moro", Pia Fondazione Cardinale G. Panico, Tricase, Lecce, Italy.
authors.forename: Rosanna
authors.initials: R
authors.lastname: Tortelli
authors.affiliation: Unit of Biostatistics, IRCCS "Casa Sollievo della Sofferenza", San Giovanni Rotondo, Foggia, Italy.
authors.forename: Massimiliano
authors.initials: M
authors.lastname: Copetti
authors.affiliation: Unit of Neurodegenerative Diseases, Department of Clinical Research in Neurology, University of Bari "Aldo Moro", Pia Fondazione Cardinale G. Panico, Tricase, Lecce, Italy.
authors.forename: Simona
authors.initials: S
authors.lastname: Arcuti
authors.affiliation: Department of Basic Medical Sciences, Neurosciences and Sense Organs, University of Bari "Aldo Moro", Bari, Italy.
authors.forename: Marianna
authors.initials: M
authors.lastname: Tursi
authors.affiliation: Department of Basic Medical Sciences, Neurosciences and Sense Organs, University of Bari "Aldo Moro", Bari, Italy.
authors.forename: Annalisa
authors.initials: A
authors.lastname: Iurillo
authors.affiliation: Unit of Neurodegenerative Diseases, Department of Clinical Research in Neurology, University of Bari "Aldo Moro", Pia Fondazione Cardinale G. Panico, Tricase, Lecce, Italy.
authors.forename: Maria Rosaria
authors.initials: MR
authors.lastname: Barulli
authors.affiliation: Department of Basic Medical Sciences, Neurosciences and Sense Organs, University of Bari "Aldo Moro", Bari, Italy.
authors.forename: Rosa
authors.initials: R
authors.lastname: Cortese
authors.affiliation: Unit of Neurodegenerative Diseases, Department of Clinical Research in Neurology, University of Bari "Aldo Moro", Pia Fondazione Cardinale G. Panico, Tricase, Lecce, Italy.
authors.forename: Rosa
authors.initials: R
authors.lastname: Capozzo
authors.affiliation: Department of Basic Medical Sciences, Neurosciences and Sense Organs, University of Bari "Aldo Moro", Bari, Italy.
authors.forename: Eustachio
authors.initials: E
authors.lastname: D'Errico
authors.affiliation: INSERM, U1094, Tropical Neuroepidemiology, Limoges, France.|CNRS FR 3503 GEIST, Tropical Neuroepidemiology, Institute of Neuroepidemiology and Tropical Neurology, UMR_S 1094, Univ. Limoges, 87000, Limoges, France.|Centre d'Epidémiologie de Biostatistique et de Méthodologie de la Recherche, CHU Limoges, Limoges, France.
authors.forename: Benoit
authors.initials: B
authors.lastname: Marin
authors.affiliation: Department of Basic Medical Sciences, Neurosciences and Sense Organs, University of Bari "Aldo Moro", Bari, Italy.
authors.forename: Isabella Laura
authors.initials: IL
authors.lastname: Simone
authors.affiliation: Unit of Neurodegenerative Diseases, Department of Clinical Research in Neurology, University of Bari "Aldo Moro", Pia Fondazione Cardinale G. Panico, Tricase, Lecce, Italy. giancarlo.logroscino@uniba.it.|Department of Basic Medical Sciences, Neurosciences and Sense Organs, University of Bari "Aldo Moro", Bari, Italy. giancarlo.logroscino@uniba.it.
authors.forename: Giancarlo
authors.initials: G
authors.lastname: Logroscino
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000690:Amyotrophic Lateral Sclerosis / Q000150:complications*; D015331:Cohort Studies; D005260:Female; D006801:Humans; D015994:Incidence; D007558:Italy / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D020828:Pseudobulbar Palsy / Q000453:epidemiology* / Q000209:etiology*; D012042:Registries; D011795:Surveys and Questionnaires
keywords: Amyotrophic lateral sclerosis; Pseudobulbar affect