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ALS Prognosis is heterogeneous across different phenotypes: a population-based study
  1. Stefano Zoccolella
  1. Department of Neurological Sciences, University of Bari,, Italy
    1. Ettore Beghi
    1. Istituto Ricerche Farmacologiche Mario Negri, Milano, Italy
      1. Guerrino Palagano
      1. Department of Neurological Sciences, University of Bari,, Italy
        1. Angela Fraddosio
        1. Department of Neurological Sciences, University of Bari,, Italy
          1. Vito Guerra
          1. Laboratory of Epidemiology, IRCCS S. De Bellis, Castellana, Bari, Italy
            1. Vito Samarelli
            1. University of Bari, Italy
              1. Vito Lepore
              1. Department of Neurological Sciences, University of Bari,, Italy
                1. Isabella Simone
                1. Department of Neurological Sciences, University of Bari,, Italy
                  1. Paolo Lamberti
                  1. Department of Neurological Sciences, University of Bari,, Italy
                    1. Luigi Serlenga
                    1. Operative Unit of Neurology, "L.Bonomo" Hospital, Andria (BA),, Italy
                      1. Giancarlo Logroscino (glogrosc{at}hsph.harvard.edu)
                      1. Department of Epidemiology HSPH, Harvard University, United States

                        Abstract

                        Background: To measure survivorship and predictors of ALS prognosis

                        Methods: Incident cases, diagnosed in the 1998-99 period and classified according to the EL Escorial criteria, were enrolled from a prospective population-based registry established in Puglia, Southern Italy, with a reference population of 4,025, 329. Cases were followed up until death or 30-June-2004.

                        Results: We identified 130 ALS incident cases while four were lost to follow-up. Median survival time was 28 months from first symptoms and 16 months from diagnosis, while cumulative survivorship at 4-years was about 30%. Advanced age (>75 years; HR:7.5; 95%CI:1.9-29.6; p=0.004) and bulbar or generalized (HR: 1.8; 95%CI:1.1-3.0; p=0.01) onset of symptoms were independent predictors of adverse survival. After stratifying patients according to site of first symptoms, age was a predictor of death among spinal (HR for patients aged >75years compared to patients aged 45 years or less: HR:11; 95%CI: 1.5-78.5; p=0.01) but not among bulbar ALS (HR: 4.5; 95%CI: 0.4-46.5; p=0.2). Among spinal onset cases, cases with predominant upper motoneuronal (UMN) involvement presented a borderline significant better survivorship (HR:0.5; 95%CI:0.2-1.3; p=0.1)

                        Conclusions: Bulbar signs and advanced age among subjects with spinal onset were indicators of poor prognosis while EEC category at entry did not predict survival. Among subjects with spinal onset of the disease, a trend for a better survivorship of subjects with UMN signs was noted.

                        • El Escorial Criteria
                        • amyotrophic lateral sclerosis
                        • prognosis
                        • survival

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