title: Incidence of amyotrophic lateral sclerosis in Rhineland-Palatinate, Germany.
abstract: There is a lack of prospective and population based epidemiological data on amyotrophic lateral sclerosis in Germany to date. The ALS registry Rhineland-Palatinate was established to investigate the incidence, course and phenotypic variety of ALS in this south-west German state of about 4 million inhabitants. During the period 2010-2011, consecutive incident patients with amyotrophic lateral sclerosis according to the revised El Escorial criteria were included and followed up using multiple overlapping sources of case ascertainment. One hundred and forty-six patients were enrolled. The annual crude incidence for amyotrophic lateral sclerosis in Rhineland-Palatinate was 1.8/100,000 person-years (95% CI 1.6-2.2). Male to female ratio was 1.1:1. Incidence increased with age reaching a peak in the 70-74 years age group and declined thereafter. Late-onset ALS (≥ 75 years) was found in 14.4% of patients. About 32% of patients presented with bulbar onset. In conclusion, incidence rate of amyotrophic lateral sclerosis in Rhineland-Palatinate is within the range of other prospective population based registers in Europe and North America. Gender ratio is nearly balanced.
authors.affiliation: Department of Neurology, Klinikum der Stadt Ludwigshafen.
authors.forename: Joachim
authors.initials: J
authors.lastname: Wolf
authors.forename: Johannes C
authors.initials: JC
authors.lastname: Wöhrle
authors.forename: Frederick
authors.initials: F
authors.lastname: Palm
authors.forename: Wilfred A
authors.initials: WA
authors.lastname: Nix
authors.forename: Matthias
authors.initials: M
authors.lastname: Maschke
authors.forename: Anton
authors.initials: A
authors.lastname: Safer
authors.forename: Heiko
authors.initials: H
authors.lastname: Becher
authors.forename: Armin J
authors.initials: AJ
authors.lastname: Grau
mesh_terms: D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D000690:Amyotrophic Lateral Sclerosis / Q000453:epidemiology*; D018672:Community Health Planning; D005260:Female; D005858:Germany / Q000453:epidemiology; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D012042:Registries; D012737:Sex Factors; D018709:Statistics, Nonparametric; D055815:Young Adult
keywords: Amyotrophic lateral sclerosis; epidemiology; incidence; population register