Johannes Dorst1, Lu Chen2, Angela Rosenbohm3, Dong-Sheng Fan2, Albert C Ludolph3, Jens Dreyhaupt4, Annemarie Hübers3, Joachim Schuster3, Jochen H Weishaupt3, Jan Kassubek3, Burkhard Gess5, Thomas Meyer6, Ute Weyen7, Andreas Hermann8, Jürgen Winkler9, Torsten Grehl10, Tim Hagenacker11, Paul Lingor12,13, Jan C Koch12, Anne Sperfeld14, Susanne Petri15, Julian Großkreutz16, Moritz Metelmann17, Joachim Wolf18, Andrea S Winkler13, Thomas Klopstock19,20,21, Matthias Boentert22, Siw Johannesen23, Alexander Storch24, Bertold Schrank25, Daniel Zeller26, Xiao-Lu Liu2, Lu Tang2. 1. Department of Neurology, Universitätsklinik Ulm, RKU, Oberer Eselsberg 45, 89081, Ulm, Germany. johannes.dorst@uni-ulm.de. 2. Department of Neurology, Peking University Third Hospital, Beijing, 100191, China. 3. Department of Neurology, Universitätsklinik Ulm, RKU, Oberer Eselsberg 45, 89081, Ulm, Germany. 4. Institute of Epidemiology and Medical Biometry, Ulm University, 89081, Ulm, Germany. 5. Department of Neurology, Uniklinik RWTH Aachen, 52074, Aachen, Germany. 6. Charité-Universitätsmedizin Berlin, 13353, Berlin, Germany. 7. Department of Neurology, BG University Hospital Bergmannsheil, 44789, Bochum, Germany. 8. Department of Neurology, University Hospital Carl Gustav Carus, Technische Universität Dresden, 01307, Dresden, Germany. 9. Department of Molecular Neurology, University Hospital Erlangen, 91054, Erlangen, Germany. 10. Department of Neurology, Alfried Krupp Hospital, 45131, Essen, Germany. 11. Department of Neurology, Essen University Hospital, 45147, Essen, Germany. 12. Department of Neurology, University Medical Center Goettingen, 37075, Göttingen, Germany. 13. Department of Neurology, Klinikum rechts der Isar der Technischen Universität München, 81675, Munich, Germany. 14. Department of Neurology, Martin Luther University of Halle-Wittenberg, 06120, Halle, Germany. 15. Department of Neurology, Hannover Medical School, 30625, Hannover, Germany. 16. Department of Neurology, Jena University Hospital, 07745, Jena, Germany. 17. Department of Neurology, Universitätsklinikum Leipzig, 04103, Leipzig, Germany. 18. Department of Neurology, Diakonissenkrankenhaus Mannheim, 68163, Mannheim, Germany. 19. Department of Neurology with Friedrich‑Baur Institute, University Hospital of the Ludwig-Maximilians-Universität München, Munich, Germany. 20. German Center for Neurodegenerative Diseases (DZNE), Munich, Germany. 21. Munich Cluster for Systems Neurology (SyNergy), Munich, Germany. 22. Department of Sleep Medicine and Neuromuscular Diseases, University Hospital Münster, 48149, Münster, Germany. 23. Department of Neurology, University Hospital of Regensburg, 93053, Regensburg, Germany. 24. Department of Neurology, University of Rostock, 18147, Rostock, Germany. 25. Department of Neurology, DKD HELIOS Klinik Wiesbaden, 65191, Wiesbaden, Germany. 26. Department of Neurology, University of Würzburg, 97080, Würzburg, Germany.
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.
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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