Yang XiangLi, Niu Lin, Yang ChunWei, Wang Lin, Liu JiXiang, He GuoYing
Department of Otolaryngology Head and Neck Surgery, Tianjin Union Medical Center, Tianjin 300121, China.
Department of Otolaryngology Head and Neck Surgery, Tianjin Union Medical Center, Tianjin 300121, China.
Am J Otolaryngol. 2019 Mar-Apr;40(2):292-296. doi: 10.1016/j.amjoto.2018.11.002. Epub 2018 Nov 5.
Myasthenia gravis (MG) is an autoimmune disease. Dysarthria, dysphagia, and difficulty swallowing as exclusive initial and primary complaints in MG (laryngeal MG) are rare and seldom reported.
Here we review and analyze the largest series of laryngeal MG patients.
A total of 30 patients with laryngeal MG as primary manifestation were found in 20 case reports/series. Dysarthria was the most frequent primary symptom (14/30), followed by dysphagia (11/30), slurred speech (4/30) and dysphonia (1/30). Sixty-three percent visited the otolaryngology department first. Only 23.33% of patients were diagnosed with MG at the first clinic visit. Forty-five percent laryngeal MG patients were acetylcholine receptor (AChR) antibody positive, 52.9% showed decremental response in the repetitive nerve stimulation (RNS) test, and 92.6% were positive in the neostigmine/edrophonium test. Fluctuating weakness was examined in 16 of 30 patients and observed in 14/16 patients.
Laryngeal MG is a rare and possibly under-diagnosed condition. The patients can present with dysarthria, dysphagia, or difficulty swallowing. Fluctuation in severity of disease by neostigmine/edrophonium test is a typical feature for MG patients. AChR antibody and RNS tests should be included to evaluate the pathologic changes in the neuromuscular junction.
重症肌无力(MG)是一种自身免疫性疾病。构音障碍、吞咽困难以及吞咽困难作为MG(喉型MG)唯一的初始和主要症状较为罕见,鲜有报道。
在此,我们回顾并分析了最大系列的喉型MG患者。
在20篇病例报告/系列研究中,共发现30例以喉型MG为主要表现的患者。构音障碍是最常见的主要症状(14/30),其次是吞咽困难(11/30)、言语不清(4/30)和发音障碍(1/30)。63%的患者首先就诊于耳鼻喉科。仅23.33%的患者在首次就诊时被诊断为MG。45%的喉型MG患者乙酰胆碱受体(AChR)抗体呈阳性,52.9%的患者在重复神经电刺激(RNS)试验中显示递减反应,92.6%的患者在新斯的明/依酚氯铵试验中呈阳性。30例患者中有16例接受了病情波动检查,其中14/16例观察到病情波动。
喉型MG是一种罕见且可能诊断不足的疾病。患者可出现构音障碍、吞咽困难或吞咽困难。新斯的明/依酚氯铵试验显示疾病严重程度的波动是MG患者的典型特征。应包括AChR抗体和RNS试验以评估神经肌肉接头的病理变化。