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非下垂型重症肌无力:一种罕见疾病的常见表现。

Non-ptotic ocular myasthenia gravis: a common presentation of an uncommon disease.

作者信息

Colavito Jennifer, Cooper Jeffrey, Ciuffreda Kenneth J

出版信息

Optometry. 2005 Jul;76(7):363-75. doi: 10.1016/j.optm.2005.05.004.

DOI:10.1016/j.optm.2005.05.004
PMID:16038863
Abstract

BACKGROUND

Myasthenia gravis (MG) is an acquired autoimmune disease of the neuromuscular junction which causes rapid muscle fatigue and weakness. Two thirds of all cases of myasthenia gravis (MG) initially manifest ptosis. In the absence of the characteristic variable ptosis, MG can present a challenge to the clinician. This article will review the current diagnostic and management strategies for MG.

CASE REPORTS

Five cases will be presented that did not initially present with ptosis. Each of these cases was previously misdiagnosed as a result of presentation of atypical myasthenia gravis signs and symptoms. The first two cases had signs and symptoms of a typical accommodative/vergence anomaly. The others manifested diplopia not normally associated with MG: one had a noncomitant vertical deviation; another had a stable 6(th) nerve palsy; and the third had a basic esotropia.

CONCLUSION

Although the hallmark findings of MG are ptosis and eye muscle palsy with variability, MG may present without ptosis, affect nonstriated muscles, and/or manifest either as a nonstrabismic vergence anomaly or as comitant nonvariable strabismic deviation.

摘要

背景

重症肌无力(MG)是一种获得性神经肌肉接头自身免疫性疾病,可导致快速的肌肉疲劳和无力。三分之二的重症肌无力(MG)病例最初表现为上睑下垂。在没有典型的可变上睑下垂的情况下,MG可能会给临床医生带来挑战。本文将综述MG目前的诊断和管理策略。

病例报告

将呈现5例最初未表现为上睑下垂的病例。这些病例中的每一例此前都因非典型重症肌无力的体征和症状而被误诊。前两例有典型的调节/聚散异常的体征和症状。其他病例表现出通常与MG无关的复视:一例有非共同性垂直偏斜;另一例有稳定的第六脑神经麻痹;第三例有基本内斜视。

结论

尽管MG的标志性表现是上睑下垂和可变的眼肌麻痹,但MG可能不伴有上睑下垂,累及非横纹肌,和/或表现为非斜视性聚散异常或共同性非可变斜视性偏斜。

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