A comparative study of myasthenic patients in the Czech and Slovak Republics

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Authors: M. Horáková ¹; I. Martinka ²; S. Voháňka ³; P. Špalek ²; J. Bednařík ³
Published in the journal: Cesk Slov Neurol N 2019; 82(2): 171-175
Category: Original Paper
doi: https://doi.org/10.14735/amcsnn2019171

Summary

Aim: Existing epidemiological studies of myasthenia feature differences in incidence, prevalence, age- and gender-specific incidences, mortality rate, and even the prevalence of myasthenia subtypes. The aim of this study was to compare the overall management of patients with myasthenia, the severity of the disease, and a number of clinical and laboratory characteristics and therapy approaches amongst patients in the Czech Republic and the Slovak Republic.

Patients and methods: Data were retrospectively collected from two registries in the Czech Republic (Brno) and the Slovak Republic (Bratislava). Data were randomly matched by age of onset of myasthenia and gender. The resulting groups consisted of 152 patients each.

Results: There was almost no significant difference between the matched groups. The clinical form, positivity of antibodies to acetylcholine receptor, Myasthenia Gravis Composite Score, time to diagnosis, and percentage of thymomas and thymectomy were not significantly different. There were significant differences only in the occurrence of limb weakness at the onset of disease and in the choice of treatment modality.

Conclusions: Our data suggest that at least part of previously observed heterogeneity between populations might be based on different age and gender distribution.

捷克和斯洛伐克共和国肌无力患者的比较研究

目标：
现有的肌无力流行病学研究表明，发病率，患病率，年龄和性别特异性发病率，死亡率，甚至是肌无力亚型的患病率存在差异。本研究的目的是比较捷克共和国和斯洛伐克共和国患者的肌无力患者的总体管理，疾病的严重程度以及临床和实验室特征以及治疗方法。

患者和方法：
从捷克共和国（布尔诺）和斯洛伐克共和国（布拉迪斯拉发）的两个登记处追溯收集数据。根据肌无力和性别的发病年龄随机匹配数据。由此产生的组由152名患者组成。

结果：
匹配组之间几乎没有显着差异。临床表现，乙酰胆碱受体抗体阳性，重症肌无力综合评分，诊断时间，胸腺瘤百分率和胸腺切除术无显着差异。仅在疾病发作时肢体无力的发生和治疗方式的选择方面存在显着差异。

结论：
我们的数据表明，至少部分先前观察到的人群之间的异质性可能基于不同的年龄和性别分布。

关键词：
重症肌无力 - 人口特征 - 比较 - 登记

Keywords:

myasthenia gravis – population characteristics – comparison – registries

Zdroje

1. Strauss AJ, Seegal BC, Hsu KC et al. Immunofluorescence demonstration of a muscle binding, complement-fixing serum globulin fraction in myasthenia gravis. Exp Biol Med 1960; 105(1): 184– 191.
2. Simpson JA. Myasthenia gravis: a new hypothesis. Scott Med J 1960; 5: 419– 436.
3. Vrolix K, Fraussen J, Molenaar PC et al. The auto-antigen repertoire in myasthenia gravis. Autoimmunity 2010; 43(5– 6): 380– 400. doi: 10.3109/ 08916930903518073.
4. Vincent A, Newsom-Davis J. Acetylcholine receptor antibody as a diagnostic test for myasthenia gravis: results in 153 validated cases and 2967 diagnostic assays. J Neurol Neurosurg Psychiatry 1985; 48(12): 1246– 1252.
5. Hoch W, McConville J, Helms S et al. Auto-antibodies to the receptor tyrosine kinase MuSK in patients with myasthenia gravis without acetylcholine receptor antibodies. Nat Med 2001; 7(3): 365– 368. doi: 10.1038/ 85520.
6. Ramanujam R, Pirskanen R, Ramanujam S et al. Utilizing twins concordance rates to infer the predisposition to myasthenia gravis. Twin Res Hum Genet 2011; 14(2): 129– 136. doi: 10.1375/ twin.14.2.129.
7. Carr AS, Cardwell CR, McCarron PO et al. A systematic review of population based epidemiological studies in myasthenia gravis. BMC Neurol 2010; 10: 46. doi: 10.1186/ 1471-2377-10-46.
8. Huang X, Liu WB, Men LN et al. Clinical features of myasthenia gravis in southern China: a retrospective review of 2,154 cases over 22 years. Neurol Sci 2013; 34(6): 911– 917. doi: 10.1007/ s10072-012-1157-z.
9. Koneczny I, Cossins J, Vincent A. The role of muscle-specific tyrosine kinase (MuSK) and mystery of MuSK myasthenia gravis. J Anat 2014; 224(1): 29– 35. doi: 10.1111/ joa.12034.
10. Štatistický úrad Slovenskej republiky. Demografia a sociálna štatistika. [online]. Available from URL: https: / / slovak.statistics.sk.
11. Martinka I, Fulova M, Spalekova M et al. Epidemiology of myasthenia gravis in Slovakia in the years 1977– 2015. Neuroepidemiology 2018; 50.
12. Český statistický úřad. Statistická ročenka Jihomoravského kraje 2015. [online]. Available from URL: https: / / www.czso.cz/ csu/ czso/ statisticka-rocenka-jihomoravskeho-kraje-2015.
13. Berrih-Aknin S, Frenkian-Cuvelier M, Eymard B. Diagnostic and clinical classification of autoimmune myasthenia gravis. J Autoimmun 2014; 48– 49: 143– 148. doi: 10.1016/ j.jaut.2014.01.003.
14. Marx A, Pfister F, Schalke B et al. The different roles of the thymus in the pathogenesis of the various myasthenia gravis subtypes. Autoimmun Rev 2013; 12(9): 875– 884. doi: 10.1016/ j.autrev.2013.03.007.
15. Pirronti T, Rinaldi P, Batocchi AP et al. Thymic lesions and myasthenia gravis. Diagnosis based on mediastinal imaging and pathological findings. Acta Radiol 2002; 43(4): 380– 384.