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迟发性全身型重症肌无力,表现为面部无力和球部症状,不累及眼外肌。

Late onset generalized myasthenia gravis presenting with facial weakness and bulbar signs without extraocular muscle involvement.

机构信息

Dipartimento di Neuroscienze, Università degli Studi di Parma, Parma, Italy.

出版信息

Neurol Sci. 2009 Aug;30(4):343-4. doi: 10.1007/s10072-009-0095-x. Epub 2009 May 30.

Abstract

Bilateral facial palsy is a common sign of autoimmune myasthenia gravis (MG). However, facial muscle weakness without clinical signs of extraocular muscle (EOM) involvement is extremely rare. We describe a late onset generalized MG presenting with facial weakness and bulbar signs without EOM deficits. The diagnosis was based on neurophysiological assessment and positive acetylcholine receptor antibody titer. Thymoma was excluded. The clinical response to corticosteroids, pyridostigmine and intravenous immunoglobulin was complete. EOM weakness was never observed during a 3-year follow-up, also during a later exacerbation of MG.

摘要

双侧面瘫是自身免疫性重症肌无力(MG)的常见征象。然而,没有眼外肌(EOM)受累临床征象的面肌无力极为罕见。我们描述了一例迟发性全身型 MG,表现为面肌无力和球部症状,无 EOM 缺损。诊断基于神经生理学评估和乙酰胆碱受体抗体滴度阳性。排除胸腺瘤。皮质类固醇、吡啶斯的明和静脉注射免疫球蛋白的临床反应完全。在 3 年的随访期间,也从未观察到 EOM 无力,即使在后来的 MG 加重期间也没有。

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