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.
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.
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.
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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Desport JC, Preux PM, Truong TC, Vallat JM, Sautereau D, Couratier P (1999) Nutritional status is a prognostic factor for survival in ALS patients. Neurology 53(5):1059–1063
Wills AM, Hubbard J, Macklin EA, Glass J, Tandan R, Simpson EP et al (2014) Hypercaloric enteral nutrition in patients with amyotrophic lateral sclerosis: a randomised, double-blind, placebo-controlled phase 2 trial. Lancet. https://doi.org/10.1016/s0140-6736(14)60222-1
Dupuis L, Corcia P, Fergani A, Gonzalez De Aguilar JL, Bonnefont-Rousselot D, Bittar R et al (2008) Dyslipidemia is a protective factor in amyotrophic lateral sclerosis. Neurology 70(13):1004–1009. https://doi.org/10.1212/01.wnl.0000285080.70324.27
Jawaid A, Salamone AR, Strutt AM, Murthy SB, Wheaton M, McDowell EJ et al (2010) ALS disease onset may occur later in patients with pre-morbid diabetes mellitus. Eur J Neurol 17(5):733–739. https://doi.org/10.1111/j.1468-1331.2009.02923.x
Chio A, Logroscino G, Traynor BJ, Collins J, Simeone JC, Goldstein LA et al (2013) Global epidemiology of amyotrophic lateral sclerosis: a systematic review of the published literature. Neuroepidemiology 41(2):118–130
Al-Chalabi A, Hardiman O (2013) The epidemiology of ALS: a conspiracy of genes, environment and time. Nat Rev Neurol 9(11):617–628
Rosenbohm A, Peter RS, Erhardt S, Lule D, Rothenbacher D, Ludolph AC et al (2017) Epidemiology of amyotrophic lateral sclerosis in Southern Germany. J Neurol 264(4):749–757
Chen L, Zhang B, Chen R, Tang L, Liu R, Yang Y et al (2015) Natural history and clinical features of sporadic amyotrophic lateral sclerosis in China. J Neurol Neurosurg Psychiatry 86(10):1075–1081
Nalini A, Thennarasu K, Gourie-Devi M, Shenoy S, Kulshreshtha D (2008) Clinical characteristics and survival pattern of 1,153 patients with amyotrophic lateral sclerosis: experience over 30 years from India. J Neurol Sci 272(1–2):60–70
Atsuta N, Watanabe H, Ito M, Tanaka F, Tamakoshi A, Nakano I et al (2009) Age at onset influences on wide-ranged clinical features of sporadic amyotrophic lateral sclerosis. J Neurol Sci 276(1–2):163–169
Rosenbohm A, Liu M, Nagel G, Peter RS, Cui B, Li X et al (2018) Phenotypic differences of amyotrophic lateral sclerosis (ALS) in China and Germany. J Neurol 265(4):774–782
Brooks BR, Miller RG, Swash M, Munsat TL (2000) El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotrop Later Scler Other Motor Neuron Disord 1(5):293–299
Cedarbaum JM, Stambler N, Malta E, Fuller C, Hilt D, Thurmond B et al (1999) The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. BDNF ALS Study Group (Phase III). J Neurol Sci 169(1–2):13–21
EdavaroneStudyGroup (2017) Safety and efficacy of edaravone in well defined patients with amyotrophic lateral sclerosis: a randomised, double-blind, placebo-controlled trial. Lancet Neurol 15(17):30111–30115
Ludolph AC, Schuster J, Dorst J, Dupuis L, Dreyhaupt J, Weishaupt JH et al (2018) Safety and efficacy of rasagiline as an add-on therapy to riluzole in patients with amyotrophic lateral sclerosis: a randomised, double-blind, parallel-group, placebo-controlled, phase 2 trial. Lancet Neurol 18(18):30175–30176
Liu X, He J, Gao FB, Gitler AD, Fan D (2018) The epidemiology and genetics of Amyotrophic lateral sclerosis in China. Brain Res 15:121–126
Wei Q, Chen X, Zheng Z, Huang R, Guo X, Cao B et al (2015) Clinical features of amyotrophic lateral sclerosis in south-west China. Amyotrop Later Scler Frontotemp Degen 16(7–8):512–519
Cui F, Liu M, Chen Y, Huang X, Cui L, Fan D et al (2014) Epidemiological characteristics of motor neuron disease in Chinese patients. Acta Neurol Scand 130(2):111–117
Zhou S, Qian S, Li X, Zheng L, Chang W, Wang L (2018) Using the capture-recapture method to estimate the incidence of amyotrophic lateral sclerosis in Beijing, China. Neuroepidemiology 50(1–2):29–34
Wais V, Rosenbohm A, Petri S, Kollewe K, Hermann A, Storch A et al (2017) The concept and diagnostic criteria of primary lateral sclerosis. Acta Neurol Scand 136(3):204–211
Pradat PF, Bruneteau G, Gordon PH, Dupuis L, Bonnefont-Rousselot D, Simon D et al. Impaired glucose tolerance in patients with amyotrophic lateral sclerosis. Amyotrophic lateral sclerosis: official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases. 2010;11(1–2):166–71. https://doi.org/10.3109/17482960902822960
Desport JC, Torny F, Lacoste M, Preux PM, Couratier P (2005) Hypermetabolism in ALS: correlations with clinical and paraclinical parameters. Neurodegener Dis 2(3–4):202–207
Bensimon G, Lacomblez L, Meininger V, ALS/Riluzole Study Group (1994) A controlled trial of riluzole in amyotrophic lateral sclerosis. N Engl J Med 330(9):585–591. https://doi.org/10.1056/nejm199403033300901
Lacomblez L, Bensimon G, Leigh PN, Guillet P, Meininger V (1996) Dose-ranging study of riluzole in amyotrophic lateral sclerosis. amyotrophic Lateral Sclerosis/Riluzole Study Group II. Lancet 347(9013):1425–1431
Zou ZY, Zhou ZR, Che CH, Liu CY, He RL, Huang HP (2017) Genetic epidemiology of amyotrophic lateral sclerosis: a systematic review and meta-analysis. J Neurol Neurosurg Psychiatry 88(7):540–549
Mariosa D, Kamel F, Bellocco R, Ye W, Fang F (2015) Association between diabetes and amyotrophic lateral sclerosis in Sweden. Eur J Neurol 22(11):1436–1442
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).
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.
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
- Amyotrophic lateral sclerosis
- Motor neuron disease
- Prognostic factors