A Toddler with Juvenile Ocular Myasthenia Gravis: Clinical Experience
DOI:
https://doi.org/10.36408/mhjcm.v8i3.679Keywords:
Ptosis, ocular, myasthenia gravis, juvenileAbstract
Background:Myasthenia gravis is an extremely rare autoimmune disorder affecting the neuromuscular junction. The incidence rate is 0.9-2.0 cases per 1 million children per year.Ocular myasthenia gravispresents as ptosis with extraocular motility restriction and is prone to be misdiagnosed as third nerve palsy and is difficult to diagnose in very young children.
Case: A girl aged 2 years 6 months with clinical features with bilateral ptosis and was diagnosed as juvenile ocular myastenia gravis based on history, physical examination and other diagnostic proceduressuch as chest X-raywithin normal limit and no thymoma, the ice test showed positive result, electromyography (EMG) showed decrement response >10%,progstigmin test showed positive result, andserum acetylcholine receptor antibody levels was 0.43 nmol/L (reference range : positive as >0.40 nmol/L).
Conclusion:Juvenile ocular myastenia gravis diagnostics can be established using simple examinations such as ice tests,prostigmin test to sophisticated examinations as systemic acetylcholinesterase antibodies. Management begins with a first-line drug, pyridostigmine, that is safe and effective. Disease monitoring and looking for etiology are very important for successful treatment.
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Sri-udomkajorn S, Panichai P, Liumsuwan S. Childhood myasthenia gravis: clinical features and outcomes. Journal of the Medical Association of Thailand =Chotmaihet thangphaet. 2011;94 Suppl 3:S152-7.
Alam MS, Devi Nivean P. Early onset bilateral juvenile myasthenia gravis masquerading as simple congenital ptosis. GMS Ophthalmol Cases. 2017;7:Doc07. Published 2017 Mar 7. doi:10.3205/oc000058
Heckmann J M, Hansen P, Toorn R van, Lubbe E, Janse van Rensburg E, Wilmshurst J. The characteristics of juvenile myasthenia gravis among South Africans. SAMJ, S. Afr. med. j. [Internet].2012 June[cited 2019 Jan 24] ; 102( 6 ): 532-536. Available from:http://www.scielo.org.za/scielo.php?script=sci_arttextpid=S0256-95742012000600070lng=en.
Nair AG, Patil-Chhablani P, Venkatramani DV, Gandhi RA. Ocular myasthenia gravis: a review. Indian J Ophthalmol. 2014;62(10):985-91.
Kubis KC, Danesh-Meyer HV, Savino PJ, Sergott RC. Theice test versus the rest test in myasthenia gravis.Ophthalmology. 2000 Nov; 107(11):1995-1998.
Miller NR, Newman NJ. The essential clinical neuroophthalmology 5th ed. Philadelphia: lippincott; 1999.
Simon RP, Aminoff MJ. Clinical neurology. Fifth ed. NewYork: Appleton and Lang; 1999.
Phillips, W. D., Vincent, A. (2016). Pathogenesis of myasthenia gravis: update on disease types, models, and mechanisms. F1000Research, 5, F1000 Faculty Rev-1513. doi:10.12688/f1000research.8206.1
Aydin, Y., Ulas, A. B., Mutlu, V., Colak, A., Eroglu, A. (2017). Thymectomy in Myasthenia Gravis. The Eurasian journal of medicine, 49(1), 48-52.
Yang WT, Lei KI, Metreweli C. Plain radiography and computed tomography of invasive thymomas: clinico-radiologic-pathologic correlation. Australasian radiology. 1997;41(2):118-24.
Huang X, Li Y, Feng H, Chen P, Liu W. Clinical Characteristics of Juvenile Myasthenia Gravis in Southern China. Frontiers in neurology. 2018;9:77.
Borenstein S, Desmedt JE. Local cooling in myasthenia. Improvement of neuromuscular failure. Archives of neurology. 1975;32(3):152-7.
Natarajan, Byju Saifudheen, Kondanath Gafoor, V Jose, James. (2016). Accuracy of the ice test in the diagnosis of myasthenic ptosis. Neurology India. 64. 1169. 10.4103/0028-3886.193780.
Tabassi, A Alireza, Dehghani Saberi, Behnam. (2005). The ice test for diagnosing myasthenia gravis. Acta Medica Iranica. 43.
Bou Ali, Hanna Salort-Campana, Emmanuelle Grapperon, Aude-Marie Gallard, Julien Franques, Jérome Sevy, Amandine Delmont, Emilien Verschueren, Annie Pouget, Jean Attarian, Shahram. (2016). New strategy for improving the diagnostic sensitivity of repetitive nerve stimulation in myasthenia gravis: RNS in MG. Muscle Nerve. 55. 10.1002/mus.25374.
Lo YL, Najjar RP, Teo KY, Tow SL, Loo JL, Milea D. A reappraisal of diagnostic tests for myasthenia gravis in a large Asian cohort. Journal of the Neurological Sciences. 2017;376:153-8.
Benatar M. A systematic review of diagnostic studies in myasthenia gravis. 2006. In: Database of Abstracts of Reviews of Effects (DARE): Quality-assessed Reviews [Internet]. York (UK): Centre for Reviews and Dissemination (UK); 1995-.
Meriggioli MN, Sanders DB. Muscle autoantibodies in myasthenia gravis: beyond diagnosis?. Expert Rev Clin Immunol. 2012;8(5):427-38.
Mehndiratta MM, Pandey S, Kuntzer T. Acetylcholinesterase inhibitor treatment for myasthenia gravis.Cochrane Database of Systematic Reviews. 2014, Issue 10. Art. No.: CD006986. DOI: 10.1002/14651858.CD006986.pub3
Sathasivam S. Current and emerging treatments for the management of myasthenia gravis. Ther Clin Risk Manag. 2011;7:313-23.
Suzuki SM, Hiroyuki I, Tomihiro N, Yuriko M, Masayuki T, Emiko K, et al. (2014). Quality of life in purely ocular myasthenia in Japan. BMC neurology. 14. 142. 10.1186/1471-2377-14-142.
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