Journal of Neurology

, Volume 265, Issue 4, pp 774–782 | Cite as

Phenotypic differences of amyotrophic lateral sclerosis (ALS) in China and Germany

  • Angela Rosenbohm
  • Mingsheng Liu
  • Gabriele Nagel
  • Raphael S. Peter
  • Bo Cui
  • Xiaoguang Li
  • Jan Kassubek
  • Dietrich Rothenbacher
  • Dorothée Lulé
  • Liying Cui
  • Albert C. Ludolph
  • For the ALS Registry Swabia Study Group
Original Communication
  • 142 Downloads

Abstract

Objective

The aim of this study is to explore phenotypical differences of amyotrophic lateral sclerosis (ALS) between two cohorts from Germany and China.

Methods

Registry-based studies of ALS were conducted in South-West Germany from 2010 to 2014 and an ALS clinic in Beijing from 2013 to 2016, respectively. Demographic and clinical features of 663 German and 276 Chinese ALS patients were collected and compared.

Results

Mean age-at-onset was higher in German than in Chinese ALS patients [66.6 years (95% CI 65.7, 67.5) vs. 53.2 years (95% CI 52.0, 54.5)]. Age distribution of ALS patients peaked around 70–74 years in Germany and 50–54 years in China. Bulbar onset was more prevalent among German than among Chinese patients (35.9 vs. 22.8%). Diagnostic delay was higher in the Chinese than in the German study sample (12 vs. 5 months). Cognitive deficits were more pronounced in the Chinese cohort. Both cohorts differed in smoking habits, prevalence of diabetes and in body mass index (BMI).

Conclusions

The apparent discrepancies between German and Chinese ALS patients (age at onset, gender distribution, bulbar forms, cognitive dysfunction, risk factors) reveal a quite different clinical phenotype in China, maybe due to socioeconomic status, environmental factors or genetic background. The observed differences in phenotype need to be pursued by further epidemiological studies on environmental and genetic risk factors.

Keywords

Amyotrophic lateral sclerosis Registry Germany China Age of onset Epidemiology 

Notes

Acknowledgements

We thank David Ewert for excellent support (figures).

This work was supported by the Deutsche Forschungsgemeinschaft (main number 577 631, project LU 336/15-1) and by the Sino-German Center (no. GZ 867). Furthermore the study was supported by the Bundesministerium für Bildung und Forschung (BMBF MND-Net) and the EU Joint Programme for Neurodegenerative Disease Research (JPND; NEEDSinALS).

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; Alber, B., 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; 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

Conflict 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 registries. The registries were approved by the Ethics Committee of Ulm University (reference 11/10) and the Research Ethics Committee of the Peking Union Medical College Hospital (PUMCH), respectively. Patients were included after informed written consent had been obtained, as set forth by the Declaration of Helsinki (WMA, 1964–2014).

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2018

Authors and Affiliations

  • Angela Rosenbohm
    • 1
  • Mingsheng Liu
    • 3
  • Gabriele Nagel
    • 2
  • Raphael S. Peter
    • 2
  • Bo Cui
    • 3
  • Xiaoguang Li
    • 3
  • Jan Kassubek
    • 1
  • Dietrich Rothenbacher
    • 2
  • Dorothée Lulé
    • 1
  • Liying Cui
    • 3
    • 4
  • Albert C. Ludolph
    • 1
  • For the ALS Registry Swabia Study Group
  1. 1.Department of NeurologyUlm University ClinicUlmGermany
  2. 2.Institute of Epidemiology and Medical BiometryUlm UniversityUlmGermany
  3. 3.Department of NeurologyPeking Union Medical College HospitalBeijingChina
  4. 4.Neuroscience CenterChinese Academy of Medical Science & Peking Union Medical CollegeBeijingChina

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