Clinical characteristics and therapeutic evaluation of childhood myasthenia gravis

Yang,Zhi‑Xiao; Xu,Kai‑Li; Xiong,Hui

doi:10.3892/etm.2015.2256

Clinical characteristics and therapeutic evaluation of childhood myasthenia gravis

Authors:
- Zhi‑Xiao Yang
- Kai‑Li Xu
- Hui Xiong
View Affiliations

Affiliations: Department of Neurology, Children's Hospital of Zhengzhou, Zhengzhou, Henan 450053, P.R. China, Department of Paediatrics, Peking University First Hospital, Beijing 100034, P.R. China
Published online on: February 3, 2015 https://doi.org/10.3892/etm.2015.2256
Pages: 1363-1368

Metrics: Total Views: 0 (Spandidos Publications: | PMC Statistics: )

Metrics: Total PDF Downloads: 0 (Spandidos Publications: | PMC Statistics: )

Cited By (CrossRef): 0 citations Loading Articles...

Abstract

This study aimed to analyze the clinical characteristics, classification and treatment of childhood myasthenia gravis (MG) and address the prognosis through follow-up. The clinical data of 135 children with MG were grouped according to clinical type and therapeutic drugs, retrospectively analyzed and prospectively monitored. Of the 135 MG patients, 85.2% had type I (ocular type), with only 4.2% progressing to systemic MG; 13.4% had type II (general type); and 1.5% had type III (fulminating type). Relapse occurred in 46.1% of the 102 patients that were followed up. The positive rate for the primary acetylcholine receptor antibody was 40.19%, without significant differences among clinical subtypes. The positive rate of the repetitive nerve stimulation frequency test by electromyography was 37.97%. Decreased expression of CD4+, CD8+, or CD3+ was present in 71% of the patients. Thymic hyperplasia was present in 5.93% of the patients, while 1.48% had thymoma. Steroid treatment was effective in the majority of the patients. Ocular type MG was common in this cohort of patients. The incidence and mortality of myasthenia crisis were low, the presence of concurrent thymoma was rare and only a limited number of children developed neurological sequelae.

View Figures

View References

1	Zouvelou V, Rentzos M, Toulas P and Evdokimidis I: AchR-positive myasthenia gravis with MRI evidence of early muscle atrophy. J Clin Neurosci. 19:918–919. 2012. View Article : Google Scholar : PubMed/NCBI
2	McGrogan A, Sneddon S and de Vries CS: The incidence of myasthenia gravis: a systematic literature review. Neuroepidemiology. 34:171–183. 2010. View Article : Google Scholar : PubMed/NCBI
3	Giraud M, Vandiedonck C and Garchon HJ: Genetic factors in autoimmune myasthenia gravis. Ann NY Acad Sci. 1132:180–192. 2008. View Article : Google Scholar : PubMed/NCBI
4	Cavalcante P, Barberis M, Cannone M, et al: Detection of poliovirus-infected macrophages in thymus of patients with myasthenia gravis. Neurology. 74:1118–1126. 2010. View Article : Google Scholar : PubMed/NCBI
5	Stefansson K, Dieperink ME, Richman DP and Marton LS: Sharing of epitopes by bacteria and the nicotinic acetylcholine receptor: a possible role in the pathogenesis of myasthenia gravis. Ann NY Acad Sci. 505:451–460. 1987. View Article : Google Scholar : PubMed/NCBI
6	Mantegazza R, Bonanno S, Camera G and Antozzi C: Current and emerging therapies for the treatment of myasthenia gravis. Neuropsychiatr Dis Treat. 7:151–160. 2011. View Article : Google Scholar : PubMed/NCBI
7	Tüzün E, Huda R and Christadoss P: Complement and cytokine based therapeutic strategies in myasthenia gravis. J Autoimmun. 37:136–143. 2011. View Article : Google Scholar : PubMed/NCBI
8	Chien PJ, Yeh JH, Chiu HC, Hsueh YM, Chen CT, Chen MC and Shih CM: Inhibition of peripheral blood natural killer cell cytotoxicity in patients with myasthenia gravis treated with plasmapheresis. Eur J Neurol. 18:1350–1357. 2011. View Article : Google Scholar : PubMed/NCBI
9	Osserman KE: Myasthenia gravis. Grune & Stratton Group; New York, NY: 1958
10	Padua L, Galassi G, Ariatti A, et al: Myasthenia gravis self-administered questionnaire: development of regional domains. Neurol Sci. 25:331–336. 2005. View Article : Google Scholar : PubMed/NCBI
11	Zhang X, Yang M, Xu J, Zhang M, Lang B, Wang W and Vincent A: Clinical and serological study of myasthenia gravis in HuBei Province, China. J Neurol Neurosurg Psychiatry. 78:386–390. 2007. View Article : Google Scholar
12	Meriggioli MN and Sanders DB: Autoimmune myasthenia gravis: emerging clinical and biological heterogeneity. Lancet Neurol. 8:475–490. 2009. View Article : Google Scholar : PubMed/NCBI
13	Shinomiya N, Nomura Y and Segawa M: A variant of childhood-onset myasthenia gravis: HLA typing and clinical characteristics in Japan. Clin Immunol. 110:154–158. 2004. View Article : Google Scholar : PubMed/NCBI
14	Spalek P, Schnorrer M and Cibulcík F: Simultaneous occurrence of acute myasthenia gravis and acute polymyositis in 3 patients and in 2 patients also associated with a thymoma. Rozhl Chir. 79:468–470. 2000.
15	Tanwani LK, Lohano V, Ewart R, Broadstone VL and Mokshagundam SP: Myasthenia gravis in conjunction with Graves’ disease: a diagnostic challenge. Endocr Pract. 7:275–278. 2001. View Article : Google Scholar : PubMed/NCBI
16	Koves IH, Cameron FJ and Kornberg AJ: Ocular myasthenia gravis and Graves disease in a 10-year-old child. J Child Neurol. 24:615–617. 2009. View Article : Google Scholar : PubMed/NCBI
17	Yoshikawa H, Satoh K, Yasukawa Y and Yamada M: Analysis of immunoglobulin secretion by lymph organs with myasthenia gravis. Acta Neurol Scand. 103:53–58. 2001. View Article : Google Scholar : PubMed/NCBI
18	Kupersmith MJ: Ocular myasthenia gravis: treatment successes and failures in patients with long-term follow-up. J Neurol. 256:1314–1320. 2009. View Article : Google Scholar : PubMed/NCBI
19	García-Carrasco M, Escárcega RO, Fuentes-Alexandro S, Riebeling C and Cervera R: Therapeutic options in autoimmune myasthenia gravis. Autoimmun Rev. 6:373–378. 2007. View Article : Google Scholar : PubMed/NCBI
20	van Sonderen A, Wirtz PW, Verschuuren JJ and Titulaer MJ: Paraneoplastic syndromes of the neuromuscular junction: therapeutic options in myasthenia gravis, Lambert-Eaton myasthenic syndrome, and neuromyotonia. Curr Treat Options Neurol. 15:224–39. 2013. View Article : Google Scholar
21	Yu L, Zhang XJ, Ma S, Li F and Zhang YF: Thoracoscopic thymectomy for myasthenia gravis with and without thymoma: a single-center experience. Ann Thorac Surg. 93:240–244. 2012. View Article : Google Scholar