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以发声障碍为主要表现的重症肌无力(MG):1520例MG患者中7例的回顾性分析

Dysphonia as a primary manifestation in myasthenia gravis (MG): a retrospective review of 7 cases among 1520 MG patients.

作者信息

Liu Wei-Bin, Xia Qiang, Men Li-na, Wu Zhong-kai, Huang Ru-xun

机构信息

Department of Neurology, the First Affiliated Hospital, Sun Yat-sen University, 58 Zhongshan 2nd Road, Guangzhou 510080, China.

出版信息

J Neurol Sci. 2007 Sep 15;260(1-2):16-22. doi: 10.1016/j.jns.2007.03.019. Epub 2007 Apr 26.

Abstract

Myasthenia gravis (MG) is an autoimmune disorder of neuromuscular transmission, usually recognized with ocular complaints or generalized muscle weakness. However, among the 1520 MG cases that had been diagnosed and treated in our hospital in the last 15 years (1990-2005), we have identified 7 MG patients whose initial and prominent complaint was dysphonia and all had been misdiagnosed elsewhere. The diagnoses were confirmed with fibrolaryngoscope and voice analysis employed before and after a positive neostigmine (anticholinesterase) test. Electromyography with repetitive stimulations, single-fiber electromyography, and laboratory and radiographic evaluations were also conducted for diagnosis. A surprisingly low seropositivity rate of anti-acetylcholine-receptor antibodies (1/7) and anti-MuSK (Muscle Specific Kinase) antibodies (0/6) were found in these dysphonia MG patients. A cholinesterase inhibitor (ChEI) and immunosuppressive therapy were applied for treatment. Extended thymectomy was applied to MG patients with thymus hyperplasia or thymic tumor. Significant improvement was found in all 7 cases after these treatments. We have developed a sere of diagnostic protocol for this rare type of laryngeal MG, and discussed the clinical implication of our data. In summary, dysphonia or laryngeal disorder can be the only prominent manifestation of MG in rare cases, which should be taken into consideration during the diagnosis to patients with exclusive laryngeal complaints.

摘要

重症肌无力(MG)是一种神经肌肉传递的自身免疫性疾病,通常以眼部症状或全身性肌肉无力为特征。然而,在我院过去15年(1990 - 2005年)诊断和治疗的1520例MG病例中,我们发现有7例MG患者最初的突出症状是发音困难,且均在其他地方被误诊。通过纤维喉镜检查以及新斯的明(抗胆碱酯酶)试验前后的语音分析确诊。还进行了重复刺激肌电图、单纤维肌电图以及实验室和影像学评估以辅助诊断。在这些发音困难型MG患者中,抗乙酰胆碱受体抗体(1/7)和抗肌肉特异性激酶(MuSK)抗体(0/6)的血清阳性率出奇的低。采用胆碱酯酶抑制剂(ChEI)和免疫抑制疗法进行治疗。对于伴有胸腺增生或胸腺瘤的MG患者实施扩大胸腺切除术。所有7例患者经这些治疗后均有显著改善。我们针对这种罕见类型的喉肌无力制定了一系列诊断方案,并讨论了我们数据的临床意义。总之,在罕见情况下,发音困难或喉部疾病可能是MG的唯一突出表现,对于仅有喉部症状的患者进行诊断时应予以考虑。

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