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Prognostic factors in ALS: a comparison between Germany and China

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Abstract

Objective

Several independent prognostic factors, such as age of onset, type of onset, body mass index (BMI), and progression rate have been identified for amyotrophic lateral sclerosis (ALS) in Caucasians. The aim of this study was to identify such factors in Chinese patients and to compare their impact with German patients.

Methods

Comparison of prognostic factors was based on two hospital-based registries. The registry of the German Network for Motor Neuron Diseases contains 3100 patients with ALS. The Chinese registry comprises 2101 patients who were collected between 2003 and 2015 in the metropolitan area of Beijing.

Results

Disease progression was slower in China [median loss of 0.50 points (IQR 0.26–0.87 points) versus 0.55 points (IQR 0.28–1.00 points) of ALS functional rating scale revised (ALS-FRS-R) score per month; p < 0.0001]. Survival of patients with ALS was similar in Germany and China (p > 0.05). We found that younger age of onset (p < 0.0001), spinal onset (p < 0.0001), high BMI (p < 0.0001) and low progression rate (p < 0.0001) were positive prognostic factors in China as well as in Germany.

Interpretation

Prognostic factors, which are known to modify the course of disease in Caucasians, apply to Chinese patients as well. The results indicate that despite the apparent differences regarding genotype and clinical phenotype, findings from interventional studies in Caucasians aiming at disease-modifying prognostic factors (such as body weight) may be transferred to Chinese patients.

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Acknowledgements

The German network for motoneuron diseases was funded by Bundesministerium für Bildung und Forschung (BMBF; 01GM1103A). Dr. Lu Chen has received funding from the National Natural Science Foundation of China (81701248). Prof. Dongsheng Fan has received funding from the National Natural Science Foundation of China (81873784).

Author information

Author notes

  1. Johannes Dorst, Lu Chen, Angela Rosenbohm, Dong-Sheng Fan and Albert C. Ludolph are contributed equally.

Authors and Affiliations

  1. Department of Neurology, Universitätsklinik Ulm, RKU, Oberer Eselsberg 45, 89081, Ulm, Germany

    Johannes Dorst, Angela Rosenbohm, Annemarie Hübers, Joachim Schuster, Jochen H. Weishaupt, Jan Kassubek & Albert C. Ludolph

  2. Department of Neurology, Peking University Third Hospital, Beijing, 100191, China

    Lu Chen, Xiao-lu Liu, Lu Tang & Dong-Sheng Fan

  3. Institute of Epidemiology and Medical Biometry, Ulm University, 89081, Ulm, Germany

    Jens Dreyhaupt

  4. Department of Neurology, Uniklinik RWTH Aachen, 52074, Aachen, Germany

    Burkhard Gess

  5. Charité–Universitätsmedizin Berlin, 13353, Berlin, Germany

    Thomas Meyer

  6. Department of Neurology, BG University Hospital Bergmannsheil, 44789, Bochum, Germany

    Ute Weyen

  7. Department of Neurology, University Hospital Carl Gustav Carus, Technische Universität Dresden, 01307, Dresden, Germany

    Andreas Hermann

  8. Department of Molecular Neurology, University Hospital Erlangen, 91054, Erlangen, Germany

    Jürgen Winkler

  9. Department of Neurology, Alfried Krupp Hospital, 45131, Essen, Germany

    Torsten Grehl

  10. Department of Neurology, Essen University Hospital, 45147, Essen, Germany

    Tim Hagenacker

  11. Department of Neurology, University Medical Center Goettingen, 37075, Göttingen, Germany

    Paul Lingor & Jan C. Koch

  12. Department of Neurology, Martin Luther University of Halle-Wittenberg, 06120, Halle, Germany

    Anne Sperfeld

  13. Department of Neurology, Hannover Medical School, 30625, Hannover, Germany

    Susanne Petri

  14. Department of Neurology, Jena University Hospital, 07745, Jena, Germany

    Julian Großkreutz

  15. Department of Neurology, Universitätsklinikum Leipzig, 04103, Leipzig, Germany

    Moritz Metelmann

  16. Department of Neurology, Diakonissenkrankenhaus Mannheim, 68163, Mannheim, Germany

    Joachim Wolf

  17. Department of Neurology, Klinikum rechts der Isar der Technischen Universität München, 81675, Munich, Germany

    Paul Lingor & Andrea S. Winkler

  18. Department of Neurology with Friedrich‑Baur Institute, University Hospital of the Ludwig-Maximilians-Universität München, Munich, Germany

    Thomas Klopstock

  19. German Center for Neurodegenerative Diseases (DZNE), Munich, Germany

    Thomas Klopstock

  20. Munich Cluster for Systems Neurology (SyNergy), Munich, Germany

    Thomas Klopstock

  21. Department of Sleep Medicine and Neuromuscular Diseases, University Hospital Münster, 48149, Münster, Germany

    Matthias Boentert

  22. Department of Neurology, University Hospital of Regensburg, 93053, Regensburg, Germany

    Siw Johannesen

  23. Department of Neurology, University of Rostock, 18147, Rostock, Germany

    Alexander Storch

  24. Department of Neurology, DKD HELIOS Klinik Wiesbaden, 65191, Wiesbaden, Germany

    Bertold Schrank

  25. Department of Neurology, University of Würzburg, 97080, Würzburg, Germany

    Daniel Zeller

Authors
  1. Johannes Dorst
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  2. Lu Chen
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  3. Angela Rosenbohm
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  4. Jens Dreyhaupt
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  5. Annemarie Hübers
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  6. Joachim Schuster
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  7. Jochen H. Weishaupt
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  8. Jan Kassubek
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  9. Burkhard Gess
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  10. Thomas Meyer
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  11. Ute Weyen
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  12. Andreas Hermann
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  13. Jürgen Winkler
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  14. Torsten Grehl
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  15. Tim Hagenacker
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  16. Paul Lingor
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  17. Jan C. Koch
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  18. Anne Sperfeld
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  19. Susanne Petri
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  20. Julian Großkreutz
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  21. Moritz Metelmann
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  22. Joachim Wolf
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  23. Andrea S. Winkler
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  24. Thomas Klopstock
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  25. Matthias Boentert
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  26. Siw Johannesen
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  27. Alexander Storch
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  28. Bertold Schrank
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  29. Daniel Zeller
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  30. Xiao-lu Liu
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  31. Lu Tang
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  32. Dong-Sheng Fan
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  33. Albert C. Ludolph
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Corresponding author

Correspondence to Johannes Dorst.

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Conflicts of interest

The German network for motoneuron diseases was funded by Bundesministerium für Bildung und Forschung (BMBF; 01GM1103A). Dr. Lu Chen has received funding from the National Natural Science Foundation of China (81701248). Prof. Dongsheng Fan has received funding from the National Natural Science Foundation of China (81873784). JDo, LC, AR, JS, AH, JDr, TM, UW, AH, JHW, JK, JW, TG, TH, PL, JCK, AS, JG, MM, JW, ASW, TK, MB, SJ, AS, BS, DZ, XL, LT, DSF, and ACL declare that they have no conflict of interest. SP reports honoraria as speaker/consultant from Biogen Idec, Novartis, Cytokinetics, TEVA Pharmaceuticals, Desitin. BG reports honoraria by Grifols, Bayer, DGN. Funding by DFG (GE 2249/1–3) and BMBF (01GM1511A).

Human participants and/or animals

The research involved human participants. The study has been approved by the local ethics committees of all participating centers and has been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.

Informed consent

All patients gave their written informed consent for data collection and analysis.

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Dorst, J., Chen, L., Rosenbohm, A. et al. Prognostic factors in ALS: a comparison between Germany and China. J Neurol 266, 1516–1525 (2019). https://doi.org/10.1007/s00415-019-09290-4

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  • DOI: https://doi.org/10.1007/s00415-019-09290-4

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