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Incidence and Geographical Variation of Amyotrophic Lateral Sclerosis (ALS) in Southern Germany – Completeness of the ALS Registry Swabia

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Uenal, Hatice, Rosenbohm, Angela, Kufeldt, Johannes, Weydt, Patrick, Goder, Katharina, Ludolph, Albert, Rothenbacher, Dietrich, Nagel, Gabriele +1 more·2014·PLoS ONE
DOI 10.1371/journal.pone.0093932PMC PMC3983245
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Abstract

Objective of this paper was to investigate the incidence, potential geographical clusters and the completeness of the amyotrophic lateral sclerosis (ALS) registry in Southern Germany (Swabia). Age-standardized incidence rates (ASR) and ratios (SIR) as well as 95% confidence intervals (CI) were estimated at county level. Capture-recapture (CARE) procedures were applied taking data source dependency into account to estimate the quality of case ascertainment in the ALS registry Swabia. We identified 438 ALS cases (53% men, 47% women) in the target population of about 8.4 Mio inhabitants. The gender ratio (men∶women) was 1.1∶1. The mean age at onset of ALS was 63.8 (SD = 11.9) years for men and 66.0 (12.2) for women. The age distribution peaked in the age group 70-74 years. The ASR of ALS was 2.5 per 100,000 person years (PY; 95% CI: 2.3-2.7). The mean SIR was 1.1 per 100,000 PY (95% CI: 1.0-1.2). High SIR suggesting geographical clusters were observed in two counties (Göppingen and Bodenseekreis), but the variation was not statistically significant (p-values = 0.2 and 0.5). The percentage of CARE estimated missing cases was 18.9% in the registry yielding an ASR of 3.1 per 100,000 PY. The high coverage of the CARE estimated completeness of the ALS registry Swabia indicates excellent quality for future projects. Regional variations have to be investigated further.

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  • The gender distribution (male-to-female ratio = 1.24:1) and gender-dependent differences in average age at symptom onset (56.8 vs 62.1 years) in the cohort were in line with values from previous ALS populations.

    “Furthermore, both the distribution of gender (male‐to‐female ratio = 1.24:1) and gender‐dependent differences in average age at symptom onset (56.8 vs. 62.1 years) were in line with values from previous ALS populations, reporting male‐to‐female ratios ranging between 0.8:1 and 2.7:1 (Alonso et al., 2009; Cronin et al., 2007; Uenal et al., 2014) and a tendency for earlier symptom‐onset in men than”

    From Prevalence of potential sports‐associated risk factors in Swiss amyotrophic lateral sclerosis patients (2017) · cites this paper as Uenal et al., 2014

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Authors
Uenal, H., Rosenbohm, A., Kufeldt, J., Weydt, P., Goder, K., Ludolph, A., Rothenbacher, D., Nagel, G., & and the ALS registry Study Group
Affiliations
  • Universität Ulm (DE)
  • University Hospital Ulm (DE)
Corresponding author
Nagel, Gabriele
ORCID
Venue
PLoS ONE
Publisher
Public Library of Science (PLoS)
Published
2014-04-10
Volume
9
Issue
4
Pages
e93932
ISSN
1932-6203 (ISSN-L)
DOI
10.1371/journal.pone.0093932
PMC
PMC3983245
OpenAlex
W2075967271
Cited by
64
References
1
Citations by year
2025: 4 · 2024: 6 · 2023: 7 · 2022: 4 · 2021: 5 · 2020: 3 · 2019: 4 · 2018: 6 · 2017: 9 · 2016: 6 · 2015: 8 · 2014: 1
FWCI
5.22
Type
journal article
Language
en
Open access
Licence
Locations
  • PLoS ONE (publishedVersion, cc-by)
  • Europe PMC (PubMed Central) (publishedVersion, cc-by)
  • PubMed Central (publishedVersion, cc-by)
  • PubMed (publishedVersion)
  • RePEc: Research Papers in Economics (submittedVersion)
  • DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion, cc-by-sa)
Funders
  • Deutsche Forschungsgemeinschaft (577631, LU 336/15‐1, project LU 336/15-1)
  • Universität Heidelberg
  • Albert-Ludwigs-Universität Freiburg
Topics
  • Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
  • Health Sciences › Medicine › Oncology › Global Cancer Incidence and Screening
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Amyloidosis: Diagnosis, Treatment, Outcomes
Keywords
amyotrophic lateral sclerosis, ALS incidence, registry completeness, mark-recapture, case ascertainment, age-standardized incidence rate, regional variation, Swabia, age of onset, sex ratio
MeSH terms
Aged, Amyotrophic Lateral Sclerosis, Data Collection, Female, Geography, Germany, Humans, Male, Middle Aged, Registries, Incidence, Cluster Analysis, Linear Models, Age of Onset, Age Distribution, Sex Distribution

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