1. Pseudobulbar affect (PBA) in an incident ALS cohort: results from the Apulia registry (SLAP)
- Author
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Tortelli, Rosa, Copetti, Massimiliano, Arcuti, Simona, Tursi, Marianna, Iurillo, Annalisa, Barulli, Rosa, Cortese, Rosa, Capozzo, Rosa, D’Errico, Eustachio, Marin, Benoît, Simone, Isabella Laura, Logroscino, Giancarlo, D'Errico, Eustachio, Università degli studi di Bari Aldo Moro (UNIBA), Statistic Unit, Istituto di Ricovero e Cura a Carattere Scientifico, Ospedale Casa Sollievo della Sofferenza [San Giovanni Rotondo] (IRCCS), Neuroépidémiologie Tropicale (NET), CHU Limoges-Institut d'Epidémiologie Neurologique et de Neurologie Tropicale-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Génomique, Environnement, Immunité, Santé, Thérapeutique (GEIST), Université de Limoges (UNILIM)-Université de Limoges (UNILIM), Laboratoire de Biostatistique et d'Informatique Médicale, Université de Limoges (UNILIM), Service de l'Information Médicale et de l'Évaluation [CHU Limoges] (SIME), and CHU Limoges
- Subjects
Male ,0301 basic medicine ,Pathology ,Neurology ,MESH: Registries ,Cohort Studies ,MESH: Aged, 80 and over ,0302 clinical medicine ,Surveys and Questionnaires ,Prospective Studies ,Registries ,MESH: Incidence ,Amyotrophic lateral sclerosis ,MESH: Cohort Studies ,MESH: Amyotrophic Lateral Sclerosis ,Neuroradiology ,Aged, 80 and over ,MESH: Aged ,education.field_of_study ,MESH: Middle Aged ,medicine.diagnostic_test ,Incidence ,Middle Aged ,3. Good health ,Italy ,Cohort ,Female ,medicine.symptom ,Adult ,medicine.medical_specialty ,Pseudobulbar affect ,Pseudobulbar Palsy ,Population ,Neurological examination ,03 medical and health sciences ,Internal medicine ,medicine ,Humans ,MESH: Surveys and Questionnaires ,education ,Pathological ,Aged ,MESH: Humans ,business.industry ,MESH: Italy ,MESH: Adult ,medicine.disease ,MESH: Male ,MESH: Prospective Studies ,MESH: Pseudobulbar Palsy ,030104 developmental biology ,[SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie ,Neurology (clinical) ,business ,MESH: Female ,030217 neurology & neurosurgery - Abstract
International audience; 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.
- Published
- 2015