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伴有破碎红纤维和乙酰胆碱受体抗体的眼外肌麻痹

External ophthalmoplegia with ragged-red fibres and acetylcholine receptor antibodies.

作者信息

Mitsikostas D, Manta P, Kalfakis N, Chioni A, Ilias A, Liakopoulos D, Papageorgiou C

机构信息

Athens National University, School of Medicine, Department of Neurology, Aeginition Hospital, Greece.

出版信息

Funct Neurol. 1995 Jul-Oct;10(4-5):209-15.

PMID:8749048
Abstract

The cases of two elderly women with external ophthalmoplegia, generalized muscle weakness and serum anti-acetylcholine receptor antibodies, are presented. The electophysiological studies showed a myopathic pattern but no indications of myasthenia after repetitive stimulation. The edrophonium test was negative and there was no response to anticholinesterase medication. In addition, elevated serum lactic acid levels and ragged-red muscle fibres in the muscle biopsy, were observed in both patients. These findings are discussed in relation to the fact that anti-acetylcholine receptor antibodies are diagnostic of myasthenia gravis, whereas ragged-red fibres and elevated lactic acid are correlated with mitochondrial myopathies.

摘要

本文报告了两例老年女性患者,她们均有眼外肌麻痹、全身肌无力以及血清抗乙酰胆碱受体抗体。电生理研究显示为肌病模式,但重复刺激后无重症肌无力迹象。依酚氯铵试验为阴性,对抗胆碱酯酶药物无反应。此外,两名患者的肌肉活检均观察到血清乳酸水平升高和破碎红纤维。鉴于抗乙酰胆碱受体抗体是重症肌无力的诊断依据,而破碎红纤维和乳酸升高与线粒体肌病相关,对这些发现进行了讨论。

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