Clinical Neurology and Neuroscience

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Juvenile Ocular Myasthenia Gravis: A Case Report

Received: Nov. 08, 2020    Accepted: Nov. 19, 2020    Published: Nov. 27, 2020
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Abstract

Myasthenia gravis is a chronic autoimmune disease of neuromuscular blockade, characterized by rapid fatigability of striated muscle. Estimated incidence has been reported at 1/200,000 to 1/1,000,000 worldwide, but its incidence in Indonesia is not clearly recorded. We report a rare case of juvenile ocular myasthenia gravis which is considered rare in child population. The objective of this case report is to describe the proper assessment of its clinical presentation. Patient is an eleven years old boy who had ptosis and opthalmoplegia on both sides for three weeks duration. Its complaint was exaggerated with sustained gaze on daily activities. Ptosis was triggered quickly after being induced by fixated upgaze without blinking and improved after ice pack was put on his eyelids for five minutes. However, his vision was preserved and no slurred speech, dysphagia or limb weakness on physical examination. Neostigmine test showed positive result and rapid nerve stimulation test also revealed withsignificant decrement on the affected eyelid muscles. No thymoma was seen on chest CT scan and thyroid function test was also normal. Hence the diagnosis was made and pyridostigmine was started for its medication. This report present a rare case of juvenile ocular myasthenia gravis. Early recognizing by some diagnostic modalities confirms its diagnosis so that treatment could be started to control the muscle weariness and improving patient's quality of life.

DOI 10.11648/j.cnn.20200404.14
Published in Clinical Neurology and Neuroscience ( Volume 4, Issue 4, December 2020 )
Page(s) 86-91
Creative Commons

This is an Open Access article, distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium or format, provided the original work is properly cited.

Copyright

Copyright © The Author(s), 2024. Published by Science Publishing Group

Keywords

Juvenile Ocular Myasthenia Gravis, Ptosis, Child

References
[1] Castro D, Derisavifard S, Anderson M, et al. Juvenile myasthenia gravis: a twenty-year experience. J ClinNeuromuscul Dis. 2013; 14: 95–102.
[2] Gilhus NE. Myasthenia gravis. N Engl J Med. 2016; 375 (26): 2570–81.
[3] Simpson JA. Myasthenia Gravis: A New Hypothesis Scottish Medical Journal 1960; Number 10.
[4] Darnindro N, Paris S, Kamelia T. Permasalahanterapipada Health Care-associated pneumonia dengan myasthenia gravis. J Indon Med Assoc. 2012; Vol 62, No: 9.
[5] Liew WKN, Kang PB. Update on juvenile myasthenia gravis. CurrOpinPediatr. 2013; 25: 694–700.
[6] Chiu HC, Vincent A, Newsom-Davis J, Hsieh KH, and Hung TP. “Myasthenia gravis: population differences in disease expression and acetylcholine receptor antibody titers between Chinese and Caucasians,” Neurology. 1987; vol. 37, no. 12, pp. 1854–57.
[7] Zhang X, Yang M, Xu J, et al. “Clinical and serological study of myasthenia gravis in HuBei Province, China,” Journal of Neurology, Neurosurgery and Psychiatry. 2007; vol. 78, no. 4, pp. 386–390.
[8] Mantegazza R, Baggi F, Antozzi C, et al. “Myasthenia gravis (MG): epidemiological data and prognostic factors,” Annals of the New York Academy of Sciences. 2003; vol. 998, pp413–23.
[9] Kalb B, Matell G, Pirskanen R, and Lambe M. “Epidemiology of myasthenia gravis: a population-based study in Stockholm, Sweden,” Neuroepidemiology. 2002; vol. 21, no. 5, pp. 221–5.
[10] Evoli A, Batocchi AP, Bartoccioni E, Lino MM, Minisci C, and Tonali P. “Juvenile myasthenia gravis with prepubertal onset,” Neuromuscular Disorders. 1998; vol. 8, no. 8, pp. 561–7.
[11] Oosterhuis HJGH, Myasthenia Gravis, Groningen Neurological Press. 1997.
[12] Parr JR, Jayawant S. “Childhood myasthenia: clinical subtypes and practical management,” Developmental Medicine and Child Neurology. 2007; vol. 49, no. 8, pp. 629–35.
[13] Ellenhorn E, Lucchese N, and Greenwald M. “Juvenile myasthenia gravis and amblyopia,” American Journal of Ophthalmology. 1986. vol. 101, no. 2, pp. 214–7.
[14] Chiang LM, Darras BT, and Kang PB. “Juvenile myasthenia gravis,” Muscle and Nerve. 2009; vol. 39, no. 4, pp. 423–31.
[15] Haliloglu G, Anlar B, Aysun S, et al. “Gender prevalence in childhood multiple sclerosis and myasthenia gravis,” Journal of Child Neurology. 2002; vol. 17, no. 5, pp. 390–2.
[16] Andrews PI, Massey JM, Howard JF, and Sanders DB. “Race, sex, and puberty influence onset, severity, and outcome in juvenile myasthenia gravis,” Neurology. 1994; vol. 44, no. 7, pp. 1208–14.
[17] Luchanok U and Kaminski HJ. “Ocular myasthenia: diagnostic and treatment recommendations and the evidence base,” Current Opinion in Neurology. 2008; vol. 21, no. 1, pp. 8–15.
[18] Bever CT, Aquino AV, Penn AS, Lovelace RE, and Rowland LP. “Prognosis of ocular myasthenia,” Annals of Neurology. 1983; vol. 14, no. 5, pp. 516-9.
[19] Pineles SL, Avery RA, Moss HE et al. “Visual and systemic outcomes in pediatric ocular myasthenia gravis,” American Journal of Ophthalmology. 2010; vol. 150, no. 4, pp. 453–9.
[20] Ortiz S and Borchert M. “Long-term outcomes of pediatric ocular myasthenia gravis,” Ophthalmology. 2008; vol. 115, no. 7, pp. 1245–8.
[21] Kim JH, Hwang JM, Hwang YS, Kim KJ, and Chae J. “Childhood ocular myasthenia gravis,” Ophthalmology. 2003; vol. 110, no. 7, pp. 1458–62.
[22] Mullaney P, Barohn RJ, Pasnoor M, McVey A, Herbelin L, Whittaker T, et al. Ocular myasthenia gravis in an academic neuro-opthalmology clinic: Clinical features and therapeutic response. J ClinNeuromuscul Dis 2011; 13: 46-52.
[23] Li ZY. China guidelines for the diagnosis and treatment of myasthenia gravis. Neuroimmunol Neuroinflammation 2016; 3: 1-9.
[24] Leite MI, Jacob S, Viegas S, et al. “IgG1 antibodies to acetylcholine receptors in “seronegative” myasthenia gravis,” Brain. 2008; vol. 131, no. 7, pp. 1940–52.
[25] Vincent A and Leite MI. “Neuromuscular junction autoimmune disease: muscle specific kinase antibodies and treatments for myasthenia gravis,” Current Opinion in Neurology. 2005; vol. 18, no. 5, pp. 519–25.
[26] Pasnoor M, Wolfe GI, Nations S, et al. “Clinical findings in MuSK-antibody positive myasthenia gravis: a U. S. experience,” Muscle and Nerve. 2010; vol. 41, no. 3, pp. 370–4.
[27] Shigemoto K. Myasthenia gravis induced by autoantibodies against MuSK. ActaMyol 2007; 26: 185-91.
[28] Hayashi A, Shiono H, Ohta M, Ohta K, Okumura M, and Sawa Y. “Heterogeneity of immunopathological features of AChR/MuSK autoantibody-negative myasthenia gravis”.
[29] Journal of Neuroimmunology. 2007; vol. 189, no. 1-2, pp. 163–8.
[30] Lindner A, Schalke B, and Toyka KV. “Outcome in juvenile onset myasthenia gravis: a retrospective study with long-term follow-up of 79 patients,” Journal of Neurology. 1997; vol. 244, no. 8, pp. 515–20.
[31] Yang ZX, Xu KL, Xiong M. Clinical characteristics and therapeutic evaluation of childhood myasthenia gravis. Exp Ther Med 2015; 9: 1363-8.
[32] Mishra S. Juvenile Myasthenia Gravis: A Short Review. Prog Asp in Pediatric & Neonat (1)-2018. PAPN. MS. ID. 000127.
[33] Grob D, Brunner N, Namba T, and Pagala M. “Lifetime course of myasthenia gravis,” Muscle and Nerve. 2008; vol. 37, no. 2, pp. 141–9.
Cite This Article
  • APA Style

    Ferry Yulianto, I Gusti Ngurah Made Suwarba, Dewi Sutriani Mahalini, Anak Agung Mas Putrawati Triningrat, Made Paramita Wijayati. (2020). Juvenile Ocular Myasthenia Gravis: A Case Report. Clinical Neurology and Neuroscience, 4(4), 86-91. https://doi.org/10.11648/j.cnn.20200404.14

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    ACS Style

    Ferry Yulianto; I Gusti Ngurah Made Suwarba; Dewi Sutriani Mahalini; Anak Agung Mas Putrawati Triningrat; Made Paramita Wijayati. Juvenile Ocular Myasthenia Gravis: A Case Report. Clin. Neurol. Neurosci. 2020, 4(4), 86-91. doi: 10.11648/j.cnn.20200404.14

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    AMA Style

    Ferry Yulianto, I Gusti Ngurah Made Suwarba, Dewi Sutriani Mahalini, Anak Agung Mas Putrawati Triningrat, Made Paramita Wijayati. Juvenile Ocular Myasthenia Gravis: A Case Report. Clin Neurol Neurosci. 2020;4(4):86-91. doi: 10.11648/j.cnn.20200404.14

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  • @article{10.11648/j.cnn.20200404.14,
      author = {Ferry Yulianto and I Gusti Ngurah Made Suwarba and Dewi Sutriani Mahalini and Anak Agung Mas Putrawati Triningrat and Made Paramita Wijayati},
      title = {Juvenile Ocular Myasthenia Gravis: A Case Report},
      journal = {Clinical Neurology and Neuroscience},
      volume = {4},
      number = {4},
      pages = {86-91},
      doi = {10.11648/j.cnn.20200404.14},
      url = {https://doi.org/10.11648/j.cnn.20200404.14},
      eprint = {https://download.sciencepg.com/pdf/10.11648.j.cnn.20200404.14},
      abstract = {Myasthenia gravis is a chronic autoimmune disease of neuromuscular blockade, characterized by rapid fatigability of striated muscle. Estimated incidence has been reported at 1/200,000 to 1/1,000,000 worldwide, but its incidence in Indonesia is not clearly recorded. We report a rare case of juvenile ocular myasthenia gravis which is considered rare in child population. The objective of this case report is to describe the proper assessment of its clinical presentation. Patient is an eleven years old boy who had ptosis and opthalmoplegia on both sides for three weeks duration. Its complaint was exaggerated with sustained gaze on daily activities. Ptosis was triggered quickly after being induced by fixated upgaze without blinking and improved after ice pack was put on his eyelids for five minutes. However, his vision was preserved and no slurred speech, dysphagia or limb weakness on physical examination. Neostigmine test showed positive result and rapid nerve stimulation test also revealed withsignificant decrement on the affected eyelid muscles. No thymoma was seen on chest CT scan and thyroid function test was also normal. Hence the diagnosis was made and pyridostigmine was started for its medication. This report present a rare case of juvenile ocular myasthenia gravis. Early recognizing by some diagnostic modalities confirms its diagnosis so that treatment could be started to control the muscle weariness and improving patient's quality of life.},
     year = {2020}
    }
    

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    T1  - Juvenile Ocular Myasthenia Gravis: A Case Report
    AU  - Ferry Yulianto
    AU  - I Gusti Ngurah Made Suwarba
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    AB  - Myasthenia gravis is a chronic autoimmune disease of neuromuscular blockade, characterized by rapid fatigability of striated muscle. Estimated incidence has been reported at 1/200,000 to 1/1,000,000 worldwide, but its incidence in Indonesia is not clearly recorded. We report a rare case of juvenile ocular myasthenia gravis which is considered rare in child population. The objective of this case report is to describe the proper assessment of its clinical presentation. Patient is an eleven years old boy who had ptosis and opthalmoplegia on both sides for three weeks duration. Its complaint was exaggerated with sustained gaze on daily activities. Ptosis was triggered quickly after being induced by fixated upgaze without blinking and improved after ice pack was put on his eyelids for five minutes. However, his vision was preserved and no slurred speech, dysphagia or limb weakness on physical examination. Neostigmine test showed positive result and rapid nerve stimulation test also revealed withsignificant decrement on the affected eyelid muscles. No thymoma was seen on chest CT scan and thyroid function test was also normal. Hence the diagnosis was made and pyridostigmine was started for its medication. This report present a rare case of juvenile ocular myasthenia gravis. Early recognizing by some diagnostic modalities confirms its diagnosis so that treatment could be started to control the muscle weariness and improving patient's quality of life.
    VL  - 4
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Author Information
  • Sanglah Hospital-Department of Pediatrics, Udayana University, Denpasar-Bali, Indonesia

  • Sanglah Hospital-Department of Pediatrics, Udayana University, Denpasar-Bali, Indonesia

  • Sanglah Hospital-Department of Pediatrics, Udayana University, Denpasar-Bali, Indonesia

  • Sanglah Hospital-Department of Ophthalmology, Udayana University, Denpasar-Bali, Indonesia

  • Sanglah Hospital-Department of Ophthalmology, Udayana University, Denpasar-Bali, Indonesia

  • Section