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Journal of Neurology

, Volume 264, Issue 4, pp 749–757 | Cite as

Epidemiology of amyotrophic lateral sclerosis in Southern Germany

  • Angela Rosenbohm
  • Raphael S. Peter
  • Siegfried Erhardt
  • Dorothée Lulé
  • Dietrich Rothenbacher
  • Albert C. Ludolph
  • Gabriele Nagel
  • The ALS Registry Study Group
Original Communication

Abstract

The objective of this study is to determine the current distribution of clinical phenotypes and to estimate future trends of ALS incidence in Western societies. We report on a clinical-epidemiological registry with a capture–recapture rate of >80% and population-based case–control study in ALS patients in South Western Germany. 1163 incidents of ALS were registered. Clinical and neuropsychological data were prospectively collected from 699 cases. The mean age at onset was 66.6 (SD = 11.6) years in prospective cases (N = 699). The site of onset was more frequently bulbar (34.1%) than lumbosacral (30.7%), cervical (27.0%), or thoracic (3.1%). Cognitive deficits (ranging from 27.5 to 42.1%, depending on the screening instrument) and behavioral changes (29%) were frequently detected. The incidence rate dropped markedly after 79 years of age, and bulbar onset as well as cognitive impairment were more frequent in ALS cases >75 years. The mean survival time of ALS cases from first paresis was 31 months. The age-standardized incidence rate (ASR) of ALS in 2012/2013 was found to be 2.4 (95% CI 2.2–2.7) per 100,000 person-years (resulting in an ASR of 3.1/100,000 with 100% coverage). Based on the predicted age distribution of the German population, the incidence of ALS was estimated to be 4.5/100,000 for men and 3.3/100,000 for women in the year 2050. ALS prevalence will rise to about 9.2–9.8/100,000 person-years in Germany in 2050. An increased proportion of patients with bulbar onset and/or cognitive deficits can be used as basic epidemiologic data on ALS for future health care decisions.

Keywords

Amyotrophic lateral sclerosis Registry Aging Cognitive impairment 

Notes

Acknowledgements

We thank P. Weydt and L. Dupuis for critical reading of the manuscript. We thank the study nurses I. Kraft-Oberbeck, N. Lämmle, and I. Dobias for their excellent field work, and the medical documentation team T. Brehme, S. Enderle, G. Feike, and B. Och for their excellent technical support. This study was supported by the German Research Council (Deutsche Forschungsgemeinschaft, DFG Grant Number LU 336/15-1).

The following cooperation partners provided data for the ALS registry Swabia:

The ALS Registry Study Group.

Andres F., Kreiskliniken Reutlingen, Department of Neurology.

Arnold G., Klinikum Sindelfingen-Böblingen, Department of Neurology.

Asshauer I., Klinikum Friedrichshafen, Department of Psychiatry and Psychotherapy.

Baezner H., Bürgerhospital Stuttgart, Department of Neurology.

Baier H., ZFP Südwürttemberg Weissenau, Department of Epileptology.

Beattie J., Ostalb-Klinikum Aalen, Department of Neurology.

Becker T., University of Ulm, Klinikum Günzburg, Department of Psychiatry and Psychotherapy II.

Behne F., ZFP Südwürttemberg Weissenau, Department of Epileptology.

Bengel D., Oberschwabenklinik Ravensburg, Department of Neurology.

Boertlein A., Bürgerhospital Stuttgart, Department of Neurology.

Bracknies, V., Klinik Dietenbronn, Department of Neurology.

Broer R., Klinikum am Weissenhof, Weinsberg, Department of Psychiatry and Psychotherapy.

Burkhard, A., Klinikum Günzburg, Department of Neurology.

Connemann B., University of Ulm, Department of Psychiatry and Psychotherapy III.

Dempewolf S., Klinikum Ludwigsburg, Department of Neurology.

Dettmers C., Schmieder Kliniken Konstanz, Department of Neurology.

Dieterich M., LMU München, Department of Neurology.

Etzersdorfer E., Furtbachkrankenhaus Stuttgart, Department of Psychiatry and Psychotherapy.

Freund, W., private practice, Biberach.

Gersner T., ZfP Zwiefalten, Department of Psychiatry and Psychotherapy.

Gold H.-J., Klinikum am Gesundbrunnen Heilbronn, Department of Neurology.

Hacke, W., University of Heidelberg, Department of Neurology.

Hamann G., Klinikum Günzburg, Department of Neurology.

Hecht M., Bezirkskrankenhaus Kaufbeuren, Department of Neurology.

Heimbach B., University of Freiburg, Department of Neurology.

Hemmer B., TU Muenchen, Department of Neurology.

Hendrich C., Klinikum Friedrichshafen, Department of Neurology.

Herting B., Diakonie-Klinikum Schwäbisch Hall, Department of Neurology.

Huber R., Klinikum Friedrichshafen, Department of Neurology.

Huber-Hartmann K., Kliniken Landkreis Heidenheim, Department of Neurology.

Hülser P.-J., Fachklinik Wangen, Department of Neurology.

Jüttler E., Ostalb-Klinikum Aalen, Department of Neurology.

Kammerer-Ciernioch J., Klinikum am Weissenhof, Weinsberg, Department of Psychiatry and Psychotherapy.

Kaspar A., Oberschwabenklinik Ravensburg, Department of Neurology.

Kern R., Klinikum Kempten, Department of Neurology.

Kimmig H., Kliniken Schwenningen, Department of Neurology.

Klebe, S., University of Würzburg, Department of Neurology.

Kloetzsch C., Schmieder Kliniken Allensbach, Department of Neurology.

Klopstock, T., LMU München, Department of Neurology.

Kohler, A., Klinikum am Gesundbrunnen Heilbronn, Department of Neurology.

Kuethmann A., Bezirkskrankenhaus Memmingen, Department of Psychiatry and Psychotherapy.

Lewis D., Marienhospital Stuttgart, Department of Neurology.

Lichy C., Klinikum Memmingen, Department of Neurology.

Lindner A., Marienhospital Stuttgart, Department of Neurology.

Mäurer M., Caritas Krankenhaus Bad Mergentheim, Department of Neurology.

Maier-Janson W., private practice, Ravensburg.

Metrikat J., Bundeswehrkrankenhaus Ulm, Department of Neurology.

Meudt O., Klinikum Memmingen, Department of Neurology.

Meyer A., Weissenau, Department of Neurology.

Müller vom Hagen J., University of Tuebingen, Department of Neurology.

Naegele A., Christophsbad Göppingen, Department of Neurology.

Naumann M., Klinikum Augsburg, Department of Neurology.

Neher K.-D., Vinzenz von Paul Hospital, Rottweil, Department of Neurology.

Neuhaus O., Kliniken Landkreis Sigmaringen, Department of Neurology.

Neusch C., private practice, Singen.

Niehaus L., Klinikum Winnenden, Department of Neurology.

Opherk C., Klinikum am Gesundbrunnen Heilbronn, Department of Neurology.

Raape J., ZFP Südwürttemberg Weissenau, Department od Neurology.

Ratzka P., Klinikum Augsburg, Department of Neurology.

Rettenmayr C., Klinikum Esslingen, Department of Neurology.

Riepe MW., Klinikum Günzburg, Department of Gerontopsychiatry.

Rothmeier J., ZFP Südwürttemberg Weissenau, Department of Neurology.

Sabolek M., Klinik Biberach, Department of Neurology.

Schabet M., Klinikum Ludwigsburg, Department of Neurology.

Schell C., Kreiskliniken Reutlingen, Department of Neurology.

Schlipf T., Klinikum Winnenden, Department of Psychiatry and Psychotherapy.

Schmauss M., Bezirkskrankenhaus Augsburg, Department of Psychiatry and Psychotherapy.

Schoels L., University of Tübingen, Department of Neurology.

Schuetz K., Kliniken Schwenningen, Department of Neurology

Schweigert B., Caritas Krankenhaus Bad Mergentheim, Department of Neurology.

Sommer N., Christophsbad Göppingen, Department of Neurology.

Sperber W., Kliniken Esslingen, Department of Neurology.

Steber C., Bezirkskrankenhaus Augsburg, Department of Psychiatry and Psychotherapy.

Steber R., Bezirkskrankenhaus Memmingen, Department of Psychiatry and Psychotherapy.

Stroick M., Klinikum Memmingen, Department of Neurology.

Synofzik, M., University of Tübingen, Deartment of Neurology.

Trottenberg T., Klinikum Winnenden, Department of Neurology.

Tumani H., Klinikum Dietenbronn, Department of Neurology.

Wahl C., Klinikum Kempten, Department of Neurology.

Weber F., Bundeswehrkrankenhaus Ulm, Department of Neurology.

Weiler M., University of Heidelberg, Department of Neurology.

Weiller C., University of Freiburg, Department of Neurology.

Wessig C., University of Würzburg, Department of Neurology.

Winkler A., TU München, Department of Neurology.

Compliance with ethical standards

Conflicts of interest

The authors declare that they have no conflict of interest.

Ethical standards

All persons gave their informed consent prior to their inclusion in the ALS Registry Swabia. The ALS Registry Swabia was approved by the local ethics committee of the University of Ulm (no. 11/10).

Supplementary material

415_2017_8413_MOESM1_ESM.tif (1.5 mb)
Supplemental Figure. County-specific incidence rates in the year 2009/2010 and 2012/2013 in the Swabian area (TIFF 1531 kb)
415_2017_8413_MOESM2_ESM.docx (15 kb)
Supplementary material 2 (DOCX 15 kb)

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Copyright information

© Springer-Verlag Berlin Heidelberg 2017

Authors and Affiliations

  • Angela Rosenbohm
    • 1
  • Raphael S. Peter
    • 2
  • Siegfried Erhardt
    • 2
  • Dorothée Lulé
    • 1
  • Dietrich Rothenbacher
    • 2
  • Albert C. Ludolph
    • 1
  • Gabriele Nagel
    • 2
  • The ALS Registry Study Group
  1. 1.Department of NeurologyUlm UniversityUlmGermany
  2. 2.Institute for Epidemiology and Medical BiometryUlm UniversityUlmGermany

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