Pseudobulbar affect (PBA) in an incident ALS cohort: results from the Apulia registry (SLAP)

Rosanna Tortelli, Massimiliano Copetti, Simona Arcuti, Marianna Tursi, Annalisa Iurillo, Maria Rosaria Barulli, Rosa Cortese, Rosa Capozzo, Eustachio D’Errico, Benoit Marin, Isabella Laura Simone, Giancarlo Logroscino

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish
Pages (from-to)316-321
Number of pages6
JournalJournal of Neurology
Volume263
Issue number2
DOIs
Publication statusPublished - Feb 1 2016

Keywords

  • Amyotrophic lateral sclerosis
  • Pseudobulbar affect

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology

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