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Controversies in Ocular Myasthenia Gravis

Myasthenia gravis (MG) with symptoms limited to eye muscles [ocular MG (OMG)] is a rare disease. OMG incidence varies according to ethnicity and age of onset. In recent years, both an increase in incidence rate, particularly in the elderly, and a lower risk for secondary generalization may have cont...

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Autores principales: Evoli, Amelia, Iorio, Raffaele
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7734350/
https://www.ncbi.nlm.nih.gov/pubmed/33329368
http://dx.doi.org/10.3389/fneur.2020.605902
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author Evoli, Amelia
Iorio, Raffaele
author_facet Evoli, Amelia
Iorio, Raffaele
author_sort Evoli, Amelia
collection PubMed
description Myasthenia gravis (MG) with symptoms limited to eye muscles [ocular MG (OMG)] is a rare disease. OMG incidence varies according to ethnicity and age of onset. In recent years, both an increase in incidence rate, particularly in the elderly, and a lower risk for secondary generalization may have contributed to the growing disease prevalence in Western countries. OMG should be considered in patients with painless ptosis and extrinsic ophthalmoparesis. Though asymmetric muscle involvement and symptom fluctuations are typical, in some cases, OMG can mimic isolated cranial nerve paresis, internuclear ophthalmoplegia, and conjugate gaze palsy. Diagnostic confirmation can be challenging in patients negative for anti-acetylcholine receptor and anti-muscle-specific tyrosine kinase antibodies on standard radioimmunoassay. Early treatment is aimed at relieving symptoms and at preventing disease progression to generalized MG. Despite the absence of high-level evidence, there is general agreement on the efficacy of steroids at low to moderate dosage; immunosuppressants are considered when steroid high maintenance doses are required. The role of thymectomy in non-thymoma patients is controversial. Prolonged exposure to immunosuppressive therapy has a negative impact on the health-related quality of life in a proportion of these patients. OMG is currently excluded from most of the treatments recently developed in generalized MG.
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spelling pubmed-77343502020-12-15 Controversies in Ocular Myasthenia Gravis Evoli, Amelia Iorio, Raffaele Front Neurol Neurology Myasthenia gravis (MG) with symptoms limited to eye muscles [ocular MG (OMG)] is a rare disease. OMG incidence varies according to ethnicity and age of onset. In recent years, both an increase in incidence rate, particularly in the elderly, and a lower risk for secondary generalization may have contributed to the growing disease prevalence in Western countries. OMG should be considered in patients with painless ptosis and extrinsic ophthalmoparesis. Though asymmetric muscle involvement and symptom fluctuations are typical, in some cases, OMG can mimic isolated cranial nerve paresis, internuclear ophthalmoplegia, and conjugate gaze palsy. Diagnostic confirmation can be challenging in patients negative for anti-acetylcholine receptor and anti-muscle-specific tyrosine kinase antibodies on standard radioimmunoassay. Early treatment is aimed at relieving symptoms and at preventing disease progression to generalized MG. Despite the absence of high-level evidence, there is general agreement on the efficacy of steroids at low to moderate dosage; immunosuppressants are considered when steroid high maintenance doses are required. The role of thymectomy in non-thymoma patients is controversial. Prolonged exposure to immunosuppressive therapy has a negative impact on the health-related quality of life in a proportion of these patients. OMG is currently excluded from most of the treatments recently developed in generalized MG. Frontiers Media S.A. 2020-11-30 /pmc/articles/PMC7734350/ /pubmed/33329368 http://dx.doi.org/10.3389/fneur.2020.605902 Text en Copyright © 2020 Evoli and Iorio. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Neurology
Evoli, Amelia
Iorio, Raffaele
Controversies in Ocular Myasthenia Gravis
title Controversies in Ocular Myasthenia Gravis
title_full Controversies in Ocular Myasthenia Gravis
title_fullStr Controversies in Ocular Myasthenia Gravis
title_full_unstemmed Controversies in Ocular Myasthenia Gravis
title_short Controversies in Ocular Myasthenia Gravis
title_sort controversies in ocular myasthenia gravis
topic Neurology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7734350/
https://www.ncbi.nlm.nih.gov/pubmed/33329368
http://dx.doi.org/10.3389/fneur.2020.605902
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