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Published on: March 13, 2018
[Treatment of pure ocular myasthenia: a proposal from Japan MG registry 2012]
1Department of Neurology, Keio University School of Medicine.
Abstract:
Autoimmune myasthenia gravis (MG), affecting both ocular and generalized muscles, results in various symptoms. Ocular myasthenia (OM) is a form of MG that is clinically restricted to extrinsic ocular muscles. Clinical signs of OM can be highly variable, ranging from mild unilateral ptosis to complete opthalmoplegia. There is no clear evidence supporting corticosteroid use for OM. We studied 123 patients whose symptoms were limited to ocular muscles with duration of illness ≥ 2 years. We classified them into two groups: 36 patients with ptosis alone (the ptosis group) and 87 with diplopia with or without ptosis (the diplopia group). Prednisolone (PSL) and immunosuppressants were less frequently used in the ptosis group. There were no significant differences in the post-treatment condition and quality-of-life (QOL) impairment between the two groups. Forty-seven (38%) patients who failed to gain minimal manifestation or better status with PSL ≤ 5 mg/day (the unfavorable condition) showed severe QOL impairment. Ocular-QMG score was a factor associated with the unfavorable condition. A treatment strategy, discriminated by ptosis or diplopia, is necessary to improve the ocular symptoms and QOL in OM patients with the unfavorable condition.
Insights
Ocular myasthenia gravis (OM) treatment strategies should differ based on ptosis or diplopia. Unfavorable outcomes and severe quality-of-life impairment were linked to the Ocular Quality-of-Life Measurement scale score.
Area of Science:
- Neurology
- Immunology
Context:
- Autoimmune myasthenia gravis (MG) affects ocular and generalized muscles.
- Ocular myasthenia (OM) specifically impacts extrinsic ocular muscles, presenting diverse symptoms from ptosis to ophthalmoplegia.
- Current evidence for corticosteroid efficacy in OM is limited.
Purpose:
- To investigate treatment strategies for ocular myasthenia (OM) based on symptom presentation (ptosis vs. diplopia).
- To identify factors associated with unfavorable treatment outcomes and quality-of-life (QOL) impairment in OM patients.
- To evaluate the effectiveness of prednisolone (PSL) and immunosuppressants in OM management.
Summary:
- A study of 123 OM patients (≥2 years duration) divided into ptosis-alone (n=36) and diplopia-with/without-ptosis (n=87) groups.
- Prednisolone (PSL) and immunosuppressants were less utilized in the ptosis group; no significant differences in post-treatment status or QOL were found between groups.
- Thirty-eight percent of patients on PSL ≤5 mg/day (unfavorable condition) experienced severe QOL impairment, with Ocular-QMG score being a significant factor.
Impact:
- Highlights the need for distinct treatment approaches for ptosis-dominant versus diplopia-dominant OM.
- Identifies Ocular-QMG score as a predictor of poor treatment response and severe QOL impairment.
- Suggests that tailored treatment strategies are crucial for improving ocular symptoms and QOL in OM patients with unfavorable prognoses.
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