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Can the age at onset give a clue to the pathogenesis of ALS?

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Abstract

Pathogenesis could play an important role in the mid- to late-life onset of symptoms in amyotrophic lateral sclerosis (ALS). An analysis of the age at onset of ALS among the incident cases occurring in the population in the Health District of Ferrara, Italy, in the period 1064–2009 was carried out. Two subsequent 23-year time intervals (1964–1986 and 1987–2009) were considered. The mean age at onset (MAAO) was estimated in relation to gender, onset type and area of residence (urban or extra-urban) at disease onset among the incident cases which occurred in the two subsequent time intervals. An uneven increase in the MAAO over time was observed as it was significant only among the female cases (from 56.7 95 % CI 51.6–61.7 years to 65.4 95 % CI 61.8–69.0 years), the overall bulbar onset cases (from 58.0 95 % CI 54.0–62.1 years to 69.3 95 % CI 66.2–72.4 years), the overall cases occurring in the extra-urban population (from 54.5 95 % CI 49.0–60.1 years to 65.1 95 % CI 60.4–69.8 years) and the bulbar onset cases occurring in the extra-urban population (from 57.1 95 % CI 53.5–60.7 years to 69.6 95 % CI 66.3–73.7 years). Although the increasing age of the population combined with improvements in ALS diagnosis among the elderly may have played a part, these uneven findings among the incident cases occurring in a well-defined homogeneous population with a stable ALS incidence would seem to suggest the involvement of risk factors associated with the extra-urban environment.

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Authors and Affiliations

  1. Neurological Clinic, biomedical and specialized surgery sciences department of the University of Ferrara, Ferrara, Italy

    Vittorio Govoni, Elena Della Coletta, Ilaria Casetta & Enrico Granieri

  2. Neurology Unit, University Hospital of Ferrara, Ferrara, Italy

    Edward Cesnik

  3. Sant’ Anna Hospital, Clinica Neurologica, Via Aldo Moro 8, 44124, Ferrara, Italy

    Vittorio Govoni

Authors
  1. Vittorio Govoni
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  2. Elena Della Coletta
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  3. Edward Cesnik
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  4. Ilaria Casetta
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  5. Enrico Granieri
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Correspondence to Vittorio Govoni.

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The authors declare that they have no conflict of interest.

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The study was performed in accordance with the ethical standards as laid down in the 1964 Declaration of Helsinki and its later amendments.

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Govoni, V., Della Coletta, E., Cesnik, E. et al. Can the age at onset give a clue to the pathogenesis of ALS?. Acta Neurol Belg 117, 221–227 (2017). https://doi.org/10.1007/s13760-016-0704-4

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  • DOI: https://doi.org/10.1007/s13760-016-0704-4

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