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用依库珠单抗成功治疗难治性全身型重症肌无力合并肌无力性不完全性眼肌麻痹

Refractory generalized myasthenia gravis with myasthenic incomplete ophthalmoplegia successfully treated with eculizumab.

作者信息

Weidmayer Sara L, Gallagher Gary

机构信息

LTC Charles S. Kettles VA Medical Center, VA Ann Arbor Healthcare System, 2215 Fuller Road, Ann Arbor, MI, United States.

WK Kellogg Eye Center, Department of Ophthalmology and Visual Sciences, University of Michigan, 1000 Wall Street, Ann Arbor, MI, United States.

出版信息

Am J Ophthalmol Case Rep. 2023 Sep 7;32:101925. doi: 10.1016/j.ajoc.2023.101925. eCollection 2023 Dec.

Abstract

PURPOSE

This is a case of myasthenic incomplete ophthalmoplegia mimicking a partial cranial nerve 3 palsy both subjectively and objectively improving after treatment with eculizumab.

OBSERVATIONS

We chronicle a case of severe generalized myasthenia gravis including myasthenia masquerading as a partial cranial nerve 3 palsy, refractory to pyridostigmine, mycophenolate, prednisone, intravenous immunoglobulin and plasma exchange but responsive to eculizumab.

CONCLUSIONS AND IMPORTANCE

This case demonstrates ocular and generalized myasthenia gravis refractory to several other therapies but amenable to eculizumab infusions, suggesting this medication may be of significant value in these difficult cases, and should be further explored for refractory ocular myasthenia gravis.

摘要

目的

这是一例重症肌无力性不完全性眼肌麻痹病例,主观和客观上均表现为类似部分动眼神经麻痹,经依库珠单抗治疗后病情改善。

观察结果

我们记录了一例严重的全身型重症肌无力病例,包括伪装成部分动眼神经麻痹的重症肌无力,对吡啶斯的明、霉酚酸酯、泼尼松、静脉注射免疫球蛋白和血浆置换均耐药,但对依库珠单抗有反应。

结论与意义

该病例表明眼肌型和全身型重症肌无力对其他几种治疗方法耐药,但对依库珠单抗输注治疗有效,提示该药物在这些疑难病例中可能具有重要价值,对于难治性眼肌型重症肌无力应进一步探索其应用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/00cf/10498161/140c3e1b242e/gr1.jpg

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