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Geographic distribution of amyotrophic lateral sclerosis through motor neuron disease mortality data

  • Neuro-Epidemiology
  • Published:
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Abstract

Amyotrophic lateral sclerosis (ALS) is a rare and devastating neurological disorder of the adult age with a prognosis of about 2–3 years from the onset of the disease. No clear cause has been identified but it seems to be a multifactorial disease with genetic and environmental components involved. Increments of mortality rates were observed since 1980 both in Italy and in many other countries. The objective of the present study is to describe the distribution of ALS mortality in Italy in the period 1980–2001 detecting single municipalities or clusters with high mortality levels for motor neuron disease (MND). ALS represents the main part (85%) of the MND group which is globally identified by the IX ICD (International Classification of Diseases and Causes of Death) 335.2 code. Death numbers and standardized mortality ratios (SMR) for MND were calculated for all Italian municipalities through the ENEA mortality database system (data source: National Institute of Statistics—ISTAT), using national mortality rates as reference. Subsequently, in order to detect municipal clusters, spatial analysis was performed. Out of the 8,099 Italian municipalities, 132 where characterized by SMR values higher than expected. Moreover 16 clusters with significant high relative risk values (RR) were identified, 12 out of them including only a single municipality. Only 22 of the municipalities with high SMR were included in the clusters. In conclusion, the two different epidemiological methodologies demonstrated to be widely complementary in detecting the geographical distribution of the disease in terms of risk for populations. A first selection of the priority areas where analytical studies should be carried on, in order to identify risk factors associated to ALS, is tentatively suggested.

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Abbreviations

ALS:

Amyotrophic lateral sclerosis

CI:

Confidence Intervals

ICD:

International Classification of Diseases

ISTAT:

Italian National Institute of Statistics

ENEA:

The National Agency for New Technologies, Energy and the Environment

LLR:

Log likelihood ratio

MND:

Motor neuron disease

OU:

Observational unit

SMR:

Standardized mortality ratio

RR:

Relative risk

s.s.:

Statistically significant

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Authors and Affiliations

  1. Section of Toxicology and Biomedical Sciences, National Agency for New Technologies, Energy and the Environment (ENEA), Rome, Italy

    Raffaella Uccelli, Pierluigi Altavista & Marina Mastrantonio

  2. Epidemiology Unit, Occupational Medicine Department, National Institute for Occupational Safety and Prevention (ISPESL), Rome, Italy

    Alessandra Binazzi

  3. Department of Environment and Primary Prevention (DACPP), Istituto Superiore di Sanità (ISS), Rome, Italy

    Stefano Belli & Pietro Comba

  4. National Centre for Epidemiology, Surveillance and Health Promotion (CNESPS), Istituto Superiore di Sanità (ISS), Rome, Italy

    Nicola Vanacore

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  1. Raffaella Uccelli
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  2. Alessandra Binazzi
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  3. Pierluigi Altavista
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  4. Stefano Belli
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  5. Pietro Comba
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  6. Marina Mastrantonio
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  7. Nicola Vanacore
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Corresponding author

Correspondence to Raffaella Uccelli.

Appendix

Appendix

Appendix 1 Municipalities with SMR values for MND significantly higher than 100 (p < 0.05) and with a number of cases ≥3 in the period 1980–2001. For each municipality number of deaths (Obs) and SMR value are indicated
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Appendix 2 Municipalities of the 4 large cluster identified by the model. Cluster number (Cluster no.), municipalities included and observed deaths (Obs) are indicated. Municipalities in bold characters are the centroids of each cluster
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Uccelli, R., Binazzi, A., Altavista, P. et al. Geographic distribution of amyotrophic lateral sclerosis through motor neuron disease mortality data. Eur J Epidemiol 22, 781–790 (2007). https://doi.org/10.1007/s10654-007-9173-7

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