Abstract：
OBJECTIVE: To clarify the clinical features of patients with Double seronegative (DS) ocular myasthenia gravis (OMG).
METHODS: Sixty-one patients diagnosed with DS OMG at the Department of Ophthalmology, Hyogo Medical University Hospital over a 5-year period from 2017 were included. Patients were classified into three groups based on the initial examination findings: group P (ptosis alone), group M (ocular motility disorder alone), and group PM (combination of both). We retrospectively reviewed the patients and clarified their clinical features.
RESULTS: There were 32 males and 29 females, with a mean age of 49.8 ± 20.9:1-82 years. Twenty-one patients (34.4%) were in group P, 23 (37.7%) in group M, and 17 (27.8%) in group PM. The proportion of males (73.9%) was significantly higher in group M compared with the other two groups. The diagnosis was proven by detection of neuromuscular junction (NMJ) disorder in 73.8%, oral pyridostigmine trial test in 13.1%, and eight patients (13.1%) in group M were diagnosed after surgical treatment. The clinical symptoms were resolved by oral pyridostigmine treatment in 54.1% of cases.
CONCLUSIONS: About 30% of patients with DS OMG had no obvious NMJ disorder, and an oral pyridostigmine trial test was necessary to diagnose these patients. Although DS OMG is often considered as the mildest form of MG, its prognosis is not optimistic and it requires aggressive therapeutic intervention.
BACKGROUND: Trial registration number: 202104-750, \"2016/4/18,\" retrospectively registered.
METHODS: Sixty-one patients diagnosed with DS OMG at the Department of Ophthalmology, Hyogo Medical University Hospital over a 5-year period from 2017 were included. Patients were classified into three groups based on the initial examination findings: group P (ptosis alone), group M (ocular motility disorder alone), and group PM (combination of both). We retrospectively reviewed the patients and clarified their clinical features.
RESULTS: There were 32 males and 29 females, with a mean age of 49.8 ± 20.9:1-82 years. Twenty-one patients (34.4%) were in group P, 23 (37.7%) in group M, and 17 (27.8%) in group PM. The proportion of males (73.9%) was significantly higher in group M compared with the other two groups. The diagnosis was proven by detection of neuromuscular junction (NMJ) disorder in 73.8%, oral pyridostigmine trial test in 13.1%, and eight patients (13.1%) in group M were diagnosed after surgical treatment. The clinical symptoms were resolved by oral pyridostigmine treatment in 54.1% of cases.
CONCLUSIONS: About 30% of patients with DS OMG had no obvious NMJ disorder, and an oral pyridostigmine trial test was necessary to diagnose these patients. Although DS OMG is often considered as the mildest form of MG, its prognosis is not optimistic and it requires aggressive therapeutic intervention.
BACKGROUND: Trial registration number: 202104-750, \"2016/4/18,\" retrospectively registered.
摘要：
目的：阐明双血清阴性（DS）眼型重症肌无力（OMG）患者的临床特征。
方法：眼科诊断为DSOMG的61例患者，包括从2017年开始的5年期间的兵库医科大学医院。根据初步检查结果将患者分为三组：P组（单纯上睑下垂），M组（单纯眼运动障碍），和组PM（两者的组合）。我们回顾性回顾了患者并阐明了他们的临床特征。
结果：男性32例，女性29例，平均年龄49.8±20.9：1-82岁。P组21例(34.4%),M组23人（37.7%），PM组17人（27.8%）。M组男性比例（73.9％）明显高于其他两组。通过检测73.8%的神经肌肉接头（NMJ）障碍来证实诊断，13.1％的口服吡啶斯的明试验，M组8例(13.1%)经手术治疗后确诊。54.1%的病例通过口服吡啶斯的明治疗可缓解临床症状。
结论：约30%的DSOMG患者无明显NMJ障碍，口服吡啶斯的明试验测试对于诊断这些患者是必要的。尽管DSOMG通常被认为是MG的最温和形式，其预后不容乐观,需要积极的治疗干预.
背景：试验登记号：202104-750，“2016/4/18”，回顾性注册。
方法：眼科诊断为DSOMG的61例患者，包括从2017年开始的5年期间的兵库医科大学医院。根据初步检查结果将患者分为三组：P组（单纯上睑下垂），M组（单纯眼运动障碍），和组PM（两者的组合）。我们回顾性回顾了患者并阐明了他们的临床特征。
结果：男性32例，女性29例，平均年龄49.8±20.9：1-82岁。P组21例(34.4%),M组23人（37.7%），PM组17人（27.8%）。M组男性比例（73.9％）明显高于其他两组。通过检测73.8%的神经肌肉接头（NMJ）障碍来证实诊断，13.1％的口服吡啶斯的明试验，M组8例(13.1%)经手术治疗后确诊。54.1%的病例通过口服吡啶斯的明治疗可缓解临床症状。
结论：约30%的DSOMG患者无明显NMJ障碍，口服吡啶斯的明试验测试对于诊断这些患者是必要的。尽管DSOMG通常被认为是MG的最温和形式，其预后不容乐观,需要积极的治疗干预.
背景：试验登记号：202104-750，“2016/4/18”，回顾性注册。
