PDF(Pubmed)
Abstract：
Myasthenia gravis (MG) is an autoimmune disorder characterized by fluctuating weakness and fatigue in ocular, bulbar, limb, or respiratory muscles. Initially, more than half of MG patients experience isolated ocular symptoms, such as ptosis, diplopia, or muscle paresis. This case report presents a unique occurrence of MG in a four-year-old female, showcasing a two-year history of sudden onset, persistent yet fluctuating unilateral ptosis accompanied by exo-deviation and adduction deficit in the right eye. No diplopia or systemic features were observed. Positive findings in tests, including the ice pack test, Cogan twitch sign, fatiguability, and neostigmine test, indicated ocular myasthenia. Electromyography revealed a decremental response, while anti-acetylcholine antibodies showed borderline results. Computed tomography of the brain ruled out central causes, and routine laboratory testing yielded normal results. Treatment with pyridostigmine and corticosteroids led to significant improvement in symptoms. This case emphasizes the diverse presentation of MG in ophthalmology, with ocular signs serving as indicators in approximately half of the cases. Early diagnosis and prompt treatment are crucial for enhancing long-term prognosis. Emergency physicians should consider MG as a potential cause for unilateral ocular symptoms after excluding central causes. Accurate diagnosis and comprehensive management of MG are complex yet essential for ensuring optimal patient health.
摘要：
重症肌无力（MG）是一种自身免疫性疾病，其特征是眼部波动无力和疲劳，球杆,肢体,或者呼吸肌.最初，超过一半的MG患者出现孤立的眼部症状，比如眼睑下垂，复视,或者肌肉麻痹.该病例报告显示了一名4岁女性的MG独特发生，展示了两年的突发病史，持续但波动的单侧上睑下垂伴有右眼的外向和内收缺陷。没有观察到复视或全身特征。测试中的阳性结果，包括冰袋测试，科根抽搐标志，可疲劳性,和新斯的明试验，指示眼肌无力。肌电图显示反应减弱，而抗乙酰胆碱抗体显示出临界结果。大脑的计算机断层扫描排除了中央原因，常规实验室检测结果正常.用吡啶斯的明和皮质类固醇治疗导致症状的显着改善。这个案例强调了MG在眼科中的多样化表现，在大约一半的病例中，眼部体征作为指标。早期诊断和及时治疗对于提高长期预后至关重要。急诊医师在排除中央原因后，应将MG视为单侧眼部症状的潜在原因。MG的准确诊断和全面管理对于确保最佳患者健康至关重要。
