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.

Key words::

• Amyotrophic lateral sclerosis
• epidemiology
• incidence
• population register

Acknowledgements

The authors are grateful to all patients who participated in the register. We particularly thank Mrs. Streib (Study nurse) and all collaborating neurologists and general practitioners.

Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.