Heterogeneity in ALSFRS-R decline and survival: a population-based study in Italy View Full Text


Ontology type: schema:ScholarlyArticle     


Article Info

DATE

2015-07-24

AUTHORS

Jessica Mandrioli, Sara Biguzzi, Carlo Guidi, Elisabetta Sette, Emilio Terlizzi, Alessandro Ravasio, Mario Casmiro, Fabrizio Salvi, Rocco Liguori, Romana Rizzi, Vladimiro Pietrini, Annamaria Borghi, Rita Rinaldi, Nicola Fini, Elisabetta Chierici, Mario Santangelo, Enrico Granieri, Vittoria Mussuto, Silvia De Pasqua, Eleni Georgoulopoulou, Antonio Fasano, ERRALS Group, Salvatore Ferro, Roberto D’Alessandro

ABSTRACT

Very few studies examined trend over time of the revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) and factors influencing it; previous studies, then, included only patients attending tertiary ALS Centres. We studied ALSFRS-R decline, factors influencing this trend and survival in a population-based setting. From 2009 onwards, a prospective registry records all incident ALS cases among residents in Emilia Romagna (population: 4.4 million). For each patient, demographic and clinical details (including ALSFRS-R) are collected by caring physicians at each follow-up. Analysis was performed on 402 incident cases (1279 ALSFRS-R assessments). The average decline of the ALSFRS-R was 0.60 points/month during the first year after diagnosis and 0.34 points/month in the second year. ALSFRS-R decline was heterogeneous among subgroups. Repeated measures mixed model showed that ALSFRS-R score decline was influenced by age at onset (p < 0.01), phenotype (p = 0.01), body mass index (BMI) (p < 0.01), progression rate at diagnosis (ΔFS) (p < 0.01), El Escorial Criteria—Revised (p < 0.01), and FVC% at diagnosis (p < 0.01). Among these factors, at multivariate analysis, only age, site of onset and ΔFS independently influenced survival. In this first population-based study on ALSFRS-R trend, we confirm that ALSFRS-R decline is not homogeneous among ALS patients and during the disease. Factors influencing ALSFRS-R decline may not match with those affecting survival. These disease modifiers should be taken into consideration for trials design and in clinical practice during discussions with patients on prognosis. More... »

PAGES

2243-2252

References to SciGraph publications

Journal

TITLE

Neurological Sciences

ISSUE

12

VOLUME

36

Author Affiliations

  • Department of Neuroscience, St. Agostino-Estense Hospital, Via Pietro Giardini n. 1355, 41100, Modena, Italy
  • Department of Neurology, Bufalini Hospital, Cesena, Italy
  • Department of Neurology, Forlì Hospital, Forlì, Italy
  • Department of Neurology, St. Anna Hospital, Ferrara, Italy
  • Department of Neurology, G. Da Saliceto Hospital, Piacenza, Italy
  • Department of Neurology, Infermi Hospital, Rimini, Italy
  • Department of Neurology, Faenza and Ravenna Hospital, Ravenna, Italy
  • Neurology Unit, Bellaria Hospital, IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy
  • Department of Biomedical and Neuromotor Sciences, University of Bologna and IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy
  • Department of Neurology, IRCCS Arcispedale Santa Maria Nuova, Reggio Emilia, Italy
  • Department of Neuroscience, Neurology Unit, University of Parma, Parma, Italy
  • Neurology Unit, Maggiore Hospital, IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy
  • Department of Neuroscience, St. Orsola-Malpighi University Hospital, Bologna, Italy
  • Department of Neurology, Fidenza Hospital, Parma, Italy
  • Department of Neurology, Carpi Hospital, Modena, Italy
  • Department of Neuroscience, University of Ferrara, Ferrara, Italy
  • Department of Neurology, Imola Hospital, Bologna, Italy
  • Department of Neuroscience, University of Modena and Reggio Emilia, Modena, Italy
  • Department of Hospital Services, Emilia Romagna Regional Health Authority, Bologna, Italy
  • Neuroepidemiology Unit, IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy
  • Identifiers

    URI

    http://scigraph.springernature.com/pub.10.1007/s10072-015-2343-6

    DOI

    http://dx.doi.org/10.1007/s10072-015-2343-6

    DIMENSIONS

    https://app.dimensions.ai/details/publication/pub.1007218603

    PUBMED

    https://www.ncbi.nlm.nih.gov/pubmed/26205535


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