Ocular Myasthenia Gravis
Keywords:
ocular myasthenia gravis, anti-acetylcholine receptor antibody, neuromuscular junctionAbstract
Ocular myasthenia gravis (OMG) is a neuromuscular disease characterized by autoantibody production against post-synaptic proteins in the neuromuscular junction. The basis, therefore, seems to be special immunological, biochemical and pathophysiological mechanisms leading to specific local changes in the neuromuscular junction. The overall incidence of OMG based on a population study is 1.13 per 100,000 per year. The diagnosis of OMG relies mainly on clinical assessment, the use of clinical tests such as the ice test, pharmacological test and electrophysiologic tests, particularly single-fiber electromyography, have a valuable role in diagnosis. The serological antibody assays for acetylcholine receptors, muscle-specific tyrosine kinase, and low-density lipoprotein 4 can be demonstrated in less than 50% of OMG. More than 20-60% of OMG patients develop generalized symptoms in the course of the disease. The treatment of OMG is primarily through cholinesterase inhibitors (pyridostigmine), immunosuppressants are frequently required in cases of ophthalmoplegia. The biomarkers are required to assess disease severity and response to therapy to optimize the management of OMG. Thus, the aim of this review is to summarize the current diagnosis, investigations, and treatment of OMG.
References
(1) Behbehani, R. Ocular Myasthenia Gravis: A Current Overview. Eye Brain. 2023; 15: 1–13.
(2) Hendricks, T. M.; Bhatti, M. T.; Hodge, D. O.; Chen, J. J. Incidence, Epidemiology, and Transformation of Ocular Myasthenia Gravis: A Population-Based Study. Am. J. Ophthalmol. 2019; 205: 99–105.
(3) Bever, C. T.; Aquino, A. V.; Penn, A. S.; Lovelace, R. E.; Rowland, L. P. Prognosis of Ocular Myasthenia. Ann. Neurol. 1983; 14(5): 516–519.
(4) Grob, D.; Brunner, N.; Namba, T.; Pagala, M. Lifetime Course of Myasthenia Gravis: Course of Myasthenia Gravis. Muscle Nerve. 2008; 37 (2):
(5) Robertson, N. P.; Deans, J.; Compston, D. A. S. Myasthenia Gravis: A Population Based Epidemiological Study in Cambridgeshire, England. J. Neurol. Neurosurg. Psychiatry. 1998; 65 (4): 492–496.
(6) Mantegazza, R.; Baggi, F.; Antozzi, C.; Confalonieri, P.; Morandi, L.; Bernasconi, P.; Andreetta, F.; Simoncini, O.; Campanella, A.; Beghi, E.; Cornelio, F. Myasthenia Gravis (MG): Epidemiological Data and Prognostic Factors. Ann. N. Y. Acad. Sci. 2003, ;998 (1): 413–423.
(7) Lazaridis, K.; Tzartos, S. J. Autoantibody Specificities in Myasthenia Gravis; Implications for Improved Diagnostics and Therapeutics. Front. Immunol. 2020;11: 212.
(8) Bandini, F.; Faga, D.; Simonetti, S. Ocular Myasthenia Mimicking a One-and-a-Half Syndrome: J. Neuroophthalmol. 2001; 21 (3): 210–211.
(9) McClard, C. K.; Lyons, L. J.; Yalamanchili, S. Bilateral Pseudo-Internuclear Ophthalmoplegia in a Patient with Myasthenia Gravis. Am. J. Ophthalmol. Case Rep. 2018; 12: 76–78.
(10) Barton, J. J.; Fouladvand, M. Ocular Aspects of Myasthenia Gravis. Semin. Neurol. 2000; Volume 20 (Number 1): 0007–0020.
(11) Averbuch-Heller, L.; Poonyathalang, A.; Von Maydell, R. D.; Remler, B. F. Hering’s Law for Eyelids: Still Valid. Neurology 1995; 45 (9): 1781–1783.
(12) Kubis, K. C.; Danesh-Meyer, H. V.; Savino, P. J.; Sergott, R. C. The Ice Test versus the Rest Test in Myasthenia Gravis. Ophthalmology 2000; 107 (11): 1995–1998.
(13) Howard, J. F. Electrodiagnosis of Disorders of Neuromuscular Transmission. Phys. Med. Rehabil. Clin. N. Am. 2013; 24 (1): 169–192.
(14) Kusner, L. L.; Puwanant, A.; Kaminski, H. J. Ocular Myasthenia: Diagnosis, Treatment, and Pathogenesis. The Neurologist 2006; 12 (5): 231–239.
(15) Costa, J.; Evangelista, T.; Conceição, I.; Carvalho, M. D. Repetitive Nerve Stimulation in Myasthenia Gravis—Relative Sensitivity of Different Muscles. Clin. Neurophysiol. 2004; 115 (12): 2776–2782.
(16) Witoonpanich, R.; Dejthevaporn, C.; Sriphrapradang, A.; Pulkes, T. Electrophysiological and Immunological Study in Myasthenia Gravis: Diagnostic Sensitivity and Correlation. Clin. Neurophysiol. 2011; 122 (9): 1873–1877.
(17) Baruca, M.; Leonardis, L.; Podnar, S.; Hojs-Fabjan, T.; Grad, A.; Jerin, A.; Blagus, R.; Šega-Jazbec, S. Single Fiber EMG as a Prognostic Tool in Myasthenia Gravis: SFEMG for MG Prognosis. Muscle Nerve 2016; 54 (6): 1034–1040.
(18) Giannoccaro, M. P.; Di Stasi, V.; Zanesini, C.; Donadio, V.; Avoni, P.; Liguori, R. Sensitivity and Specificity of Single-Fibre EMG in the Diagnosis of Ocular Myasthenia Varies Accordingly to Clinical Presentation. J. Neurol. 2020; 267 (3): 739–745.
(19) Hoch, W.; McConville, J.; Helms, S.; Newsom-Davis, J.; Melms, A.; Vincent, A. Auto-Antibodies to the Receptor Tyrosine Kinase MuSK in Patients with Myasthenia Gravis without Acetylcholine Receptor Antibodies. Nat. Med. 2001; 7 (3): 365–
(20) Bau, V.; Hanisch, F.; Hain, B.; Zierz, S. Okuläre Beteiligung bei MuSK-Antikörper-positiver Myasthenia gravis. Klin. Monatsblätter Für Augenheilkd. 2006; 223 (1): 81–83.
(21) Higuchi, O.; Hamuro, J.; Motomura, M.; Yamanashi, Y. Autoantibodies to Low-Density Lipoprotein Receptor-Related Protein 4 in Myasthenia Gravis. Ann. Neurol. 2011; 69 (2): 418–422.
(22) Zouvelou, V.; Zisimopoulou, P.; Rentzos, M.; Karandreas, N.; Evangelakou, P.; Stamboulis, E.; Tzartos, S. J. Double Seronegative Myasthenia Gravis with Anti-LRP 4 Antibodies. Neuromuscul. Disord. 2013; 23 (7): 568–570.
(23) Tsivgoulis, G.; Dervenoulas, G.; Kokotis, P.; Zompola, C.; Tzartos, J. S.; Tzartos, S. J.; Voumvourakis, K. I. Double Seronegative Myasthenia Gravis with Low Density Lipoprotein-4 (LRP4) Antibodies Presenting with Isolated Ocular Symptoms. J. Neurol. Sci. 2014; 346 (1–2): 328–330.
(24) Amin, S.; Aung, M.; Gandhi, F. R.; Pena Escobar, J. A.; Gulraiz, A.; Malik, B. H. Myasthenia Gravis and Its Association With Thyroid Diseases. Cureus 2020.
(25) Treatment of Ocular Myasthenia Gravis. Asia-Pac. J. Ophthalmol. 2019.
(26) Shah, Y. S.; Henderson, A. D.; Carey, A. R. Effect of Initial Prednisone Dosing on Ocular Myasthenia Gravis Control. J. Neuroophthalmol. 2021; 41 (4): e622–e626.
(27) Threetong, T.; Poonyathalang, A.; Preechawat, P.; Jindahra, P.; Padungkiatsagul, T.; Vanikieti, K. Initial Treatment Response in Ocular Myasthenia Gravis: A Comparison Between Low and Moderate Doses of Prednisolone. Clin. Ophthalmol. 2020: 14: 2051–2056.
(28) Bentley, C. R.; Dawson, E.; Lee, J. P. Active Management in Patients with Ocular Manifestations of Myasthenia Gravis. Eye 2001; 15 (1): 18–22.
(29) Hendricks, T. M.; Bhatti, M. T.; Hodge, D. O.; Chen, J. J. Incidence, Epidemiology, and Transformation of Ocular Myasthenia Gravis: A Population-Based Study. Am. J. Ophthalmol. 2019; 205: 99–105.
(30) Galassi, G.; Mazzoli, M.; Ariatti, A.; Kaleci, S.; Valzania, F.; Nichelli, P. F. Antibody Profile May Predict Outcome in Ocular Myasthenia Gravis. Acta Neurol. Belg. 2018; 118 (3): 435–443.
(31) Supawongwattana, M.; Vanikieti, K.; Jindahra, P.; Padungkiatsagul, T. Significance of Acetylcholine Receptor Antibody Titers in Acetylcholine Receptor Antibody-Positive Ocular Myasthenia Gravis: Generalization and Presence of Thyroid Autoimmune Antibodies and Thymoma. Clin. Ophthalmol. 2023; 17: 649–656.
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