#PAGE_PARAMS# #ADS_HEAD_SCRIPTS# #MICRODATA#

Ocular Myasthenia Gravis in Slovak Republic


Authors: I. Martinka 1;  F. Cibulčík 1;  J. Bednařík 2;  P. Špalek 1
Authors‘ workplace: Centrum pre neuromuskulárne ochorenia, Neurologická klinika SZU a UN Bratislava 1;  Neurologická klinika LF MU a FN Brno 2
Published in: Cesk Slov Neurol N 2017; 80/113(2): 190-196
Category: Original Paper
doi: https://doi.org/10.14735/amcsnn2017190

Overview

Background:
Ocular symptoms are the most common initial symptoms of myasthenia gravis (MG) and they generalize rapidly in the majority of MG patients. In purely ocular MG (OMG), impairment of neuromuscular transmission remains limited to extraocular muscles even after three years from OMG onset.

Aim:
We performed retrospective longitudinal study in 946 MG patients with initial ocular symptoms (oMG) registered between 1978 and 2015 at the Slovak Centre for Neuromuscular Diseases. The aim of this study was to analyze clinical assessments, laboratory findings and prognostic factors in oMG.

Material and methods:
We analyzed data and findings in medical records of oMG patients including age at onset, sex, autoantibodies against acetylcholine receptors (AChRs) and muscle-specific kinase (MuSK), a type of clinical symptomatology. We evaluated the therapies used, clinical status at the last examination and prognosis in this initial clinical pattern.

Results:
Impairment of ocular muscles as the first MG sign occurred in 946 patients (M = 487, F = 459). oMG generalized in due course in 554 (58.6%) patients. We identified male gender (RR 1.23), age at onset of over 50 years (RR 1.23), association with other autoimmune diseases (RR 1.57), AChR - seropositivity (RR 2.09) and thymoma (RR 15.71) as the risk factors of oMG generalization. We identified female gender (RR 1.2), AChR - seronegativity (RR 1.58), oMG generalization (RR 1.63) and age at onset of below 50 years (RR 2.08) as predictive factors of unfavourable outcome (repeated exacerbations, unfavourable post-intervention status).

Conclusion:
To improve oMG prognosis, it is important to recognize the oMG patients with high risk of generalization and/or unfavourable outcome and to select appropriate therapy for these patients.

Key words:
myasthenia gravis – ocular myasthenia gravis – diagnosis – prognosis – generalization

The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study.

The Editorial Board declares that the manuscript met the ICMJE “uniform requirements” for biomedical papers.


Chinese summary - 摘要

斯洛伐克共和国眼科重症肌无力

背景


眼部症状通常是重症肌无力(MG)的最初表现,并且它们在大多数MG患者中发展的很迅速。在纯粹的眼部MG(OMG)中,即使在OMG发病三年后,仍然存在有限的存在损伤的神经肌肉传播到眼外肌。

目标

我们对1978年至2015年间在斯洛伐克神经肌肉疾病中心登记的946名MG患者进行了追溯性纵向研究。本研究的目的是分析oMG中的临床评估,实验室检查结果和预后因素。

材料和方法

我们分析了oMG患者的病历数据,包括发病年龄,性别,乙酰胆碱受体(AChR)和肌肉特异性激酶(MuSK)的一种临床症状学类型。我们评估了使用的治疗方法,最后一次检查时的临床状态和初步临床模式的预后。

结果

946例MG患者均首次出现了眼肌损伤的征兆(男 = 487,女 = 459)。 oMG在554例(58.6%)患者中适时推广。我们确定男性性别(RR 1.23),50岁以上发病(RR 1.23),与其他自身免疫性疾病(RR 1.57),AChR-血清阳性(RR 2.09)和胸腺瘤(RR 15.71)的关系作为oMG的危险因素。我们确定女性性别(RR 1.2),AChR - 血清反应性(RR 1.58),oMG泛化(RR 1.63)和50岁以下发病(RR 2.08)作为不良后果的预测因素(重复加重,不良干预后状态)。

结论

为了改善oMG预后,重要的是要识别具有广泛性风险和/或不利后果的oMG患者,并为这些患者选择合适的治疗方法。

关键词

重症肌无力 - 重症肌无力 - 诊断 - 预后 - 泛化


Sources

1. Špalek P, Cibulčík F. Okulárna myasténia gravis –  dia­gnostika, liečba, prognóza. [as­ses­sed 2009]. Dostupné z URL: http:/ / www.myastheniagravis.cz/ images/ spalek09.pdf.

2. Kerty E, Elsais A, Argov Z, et al. EFNS/ ENS Guidelines for the treatment of ocular myasthenia. Eur J Neurol 2014;21(5):687– 93. doi: 10.1111/ ene.12359.

3. Grob D, Arsura EL, Brun­ner NG, et al. The course of myasthenia gravis and therapies af­fect­ing outcome. Ann NY Acad Sci 1987;505:472– 99.

4. Grob D, Brun­ner N, Namba T, et al. Lifetime course of myasthenia gravis. Muscle Nerve 2008;37(2):141– 9.

5. Kupersmith MJ, Latkany R, Homel P. Development of generalized disease at 2 years in patients with ocular myasthenia gravis. Arch Neurol 2003;60(2):243– 8.

6. Luchanok V, Kaminski HJ. Natural history of myasthenia gravis. In: Engel AG, ed. Myasthenia gravis and myasthenic disorders. 2nd ed. Oxford University Pres­s, New York 2012:93– 4.

7. Wong SH, Huda S, Vincent A, et al. Ocular myasthenia gravis: controversies and updates. Curr Neurol Neurosci Rep 2014;14(1):421. doi: 10.1007/ s11910-013-0421-9.

8. Kupersmith MJ. Ocular myasthenia gravis: treatment succes­ses and failures in patients with long-term fol­low-up. J Neurol 2009;256(8):1314– 20. doi: 10.1007/ s00415-009-5120-8.

9. Pitz S, Jordan B, Zierz S. Okuläre myasthenie. Ophthalmologe 2013;110(12):1202.

10. Nair AG, Patil-Chhablani AG, Venkatramani DV, et al. Ocular myasthenia gravis: a review. Indian J Ophthalmol 2014;62(10):985– 91. doi: 10.4103/ 0301-4738.145987.

11. Jaretzki A, Barohn RJ, Ernstoff RM. Myasthenia gravis: recom­mendations for clinical research standards. Task force of the medical scientific advisory board of the Myasthenia gravis foundation of America. Neurology 2000;55(1):327– 34.

12. Suzuki S, Murai H, Imai T, et al. Quality of life in purely ocular myasthenia in Japan. BMC Neurol 2014;14:142. doi: 10.1186/ 1471-2377-14-142.

13. Roh HS, Lee SY, Yoon JS. Comparison of clinical manifestations between patients with ocular myasthenia gravis and generalized myasthenia gravis. Korean J Ophtalmol 2011;25(1):1– 7. doi: 10.3341/ kjo.2011.25.1.1.

14. Hong YH, Kwon SB, Kim BJ, et al. Prognosis of ocular myasthenia in Korea: a retrospective multicenter analysis of 202 patients. J Neurol Sci 2008;273(1– 2):10– 4. doi: 10.1016/ j.jns.2008.05.023.

15. Kamada M, Matsui N, Sakai W, et al. Ocular myasthenia gravis with anti-muscle-specific tyrosine kinase antibodies: two new cases and systematic literature review. Clin Experiment Neurol. In Press 2016. doi: 10.1111/ cen3.12296.

16. Lee JJ, Koh KM, Kim US. The anti-acetylcholine receptor antibody test in suspected ocular myasthenia gravis. J Ophtalmol 2014;2014:68979. doi: 10.1155/ 2014/ 689792.

17. Peeler CE, De Lott LB, Nagia L, et al. Clinical utility of acetylcholine receptor antibody test­ing in ocular myasthenia gravis. JAMA Neurol 2015;72(10):1170– 4. doi: 10.1001/ jamaneurol.2015.1444.

18. MacLen­nan C, Beeson D, Buijs AM, et al. Acetylcholine receptor expres­sion in human extraocular muscles and their susceptibility to myasthenia gravis. Ann Neurol 1997;41(4):423– 31.

19. Evoli A, Batocchi AP, Minisci C, et al. Therapeutic options in ocular myasthenia gravis. Neuromuscul Disord 2001;11(2):208– 16.

20. Gilbert ME, De Sousa EA, Savino PJ. Ocular myasthenia gravis treatment: the case against prednisone ther­apy and thymectomy. Arch Neurol 2007;64(12):1790– 2.

21. Kaminski HJ, Daroff RB. Treatment of ocular myasthenia: steroids only when compel­led. Arch Neurol 2000;57(5):752– 3.

22. Roach ES. Treat­ing ocular Myasthenia Gravis with inadequate evidence. Arch Neurol 2007;64(12):1794– 5.

23. Benatar M, McDermott MP, Sanders DB, et al. Ef­ficacy of prednisone for the treatment of ocular myasthenia (EPITOME): a randomized, control­led trial. Muscle Nerve 2016;53(3):363– 9. doi: 10.1002/ mus.24769.

24. Mee J, Paine M, Byrne E, et al. Im­munotherapy of ocular myasthenia gravis reduces conversion to generalized myasthenia gravis. J Neuroophthalmol 2003;23(4):251– 5.

25. Monsul NT, Patwa HS, Knorr AM, et al. The ef­fect of prednisone on the progres­sion from ocular to generalized myasthenia gravis. J Neurol Sci 2004;217(2):131– 3.

26. Lanska DJ. Indications for thymectomy in myasthenia gravis. Neurology 1990;40(12):1828– 9.

27. Liu Z, Feng H, Yeung SJ, et al. Extended trans­sternal thymectomy for the treatment of ocular myasthenia gravis. Ann Thorac Surg 2011;92(6):1993– 9. doi: 10.1016/ j.athoracsur.2011.08.001.

28. Bever CT, Aquino AV, Penn AS, et al. Prognosis of ocular myasthenia. Ann Neurol 1983;14(5):516– 9.

29. Al­len JA, Scala S, Jones HR. Ocular myasthenia gravis in a senior population, dia­gnosis, therapy and prognosis. Muscle Nerve 2010;41(3):379– 84. doi: 10.1002/ mus.21555.

30. Guan YZ, Cui LY, Liu MS, et al. Progres­sion from ocular to generalized myasthenia gravis in adults. Chin J Neurol 2012;45(12):866– 8.

31. Nagia L, Lemos J, Abusamra K. Prognosis of ocular myasthenia gravis. Retrospective multicenter analysis. Ophthalmology 2015;122(7):1517– 21. doi: 10.1016/ j.ophtha.2015.03.010.

32. Wong SH, Petrie A, Plant GT. Ocular Myasthenia gravis: toward a risk of generalization score and sample size calculation for a randomized control­led trial of disease modification. J Neuroophthalmol 2016;36(3):252– 8. doi: 10.1097/ WNO.0000000000000350.

33. Hong YH, Lee KW, Sung JJ, et al. Prognostic factors of secondary generalization in patients with ocular myasthenia gravis: a retrospective single hospital-based study. Neuromusc Dis 2006;16:117.

34. Ariatti A, Stefani M, Miceli P, et al. Prognostic factors and health-related quality of life in ocular myasthenia gravis (OMG). Int J Neurosc 2014;124(6):427– 35. doi: 10.3109/ 00207454.2013.853664.

35. Wang LL, Zhang Y, He ML. Clinical features and prognosis of ocular myasthenia gravis patients with dif­ferent phenotypes. Chin Med J 2015;128(19):2682– 4. doi: 10.4103/ 0366-6999.166032.

36. Romi F, Gilhus NE, Aarli JA. Myasthenia gravis: dis­ease severity and prognosis. Acta Neurol Scand 2006;113(Suppl 183):24– 5.

Labels
Paediatric neurology Neurosurgery Neurology

Article was published in

Czech and Slovak Neurology and Neurosurgery

Issue 2

2017 Issue 2

Most read in this issue
Login
Forgotten password

Enter the email address that you registered with. We will send you instructions on how to set a new password.

Login

Don‘t have an account?  Create new account

#ADS_BOTTOM_SCRIPTS#