Hong Yoon-Ho, Kwon Seok-Beom, Kim Byung-Jo, Kim Byoung Joon, Kim Seung Hyun, Kim Jong Kuk, Park Kyung-Seok, Park Ki-Jong, Sung Jung-Joon, Sohn Eun Hee, Lee Yeong-Bae, Jeong Dushin, Joo In Soo, Choi Byung-Ok, Choi Young-Chul
Seoul National University Boramae Hospital, South Korea.
J Neurol Sci. 2008 Oct 15;273(1-2):10-4. doi: 10.1016/j.jns.2008.05.023. Epub 2008 Jul 3.
The aims of this study were to obtain data on the frequency with which Korean patients with autoimmune myasthenia gravis (MG) present solely with ocular disturbances and progress to develop generalized disease and to identify the prognostic factors associated with secondary generalization.
We conducted a retrospective multicenter survey in which a total of 376 adult patients who were newly diagnosed with MG from 2000 through 2005 were reviewed for analysis. Patients with ocular MG at the time of symptom presentation (n=202, 53.7%) were divided into two subgroups according to their prognosis: the patients whose disease remained ocular throughout the follow-ups were placed in the OMG-R group, and the patients who progressed to develop generalized disease were placed in the OMG-G group. Clinical characteristics and laboratory findings were compared between the two subgroups.
Secondary generalization developed in 47 (23.3%) of the 202 study subjects, mostly within the first 6 months after symptom presentation, while the disease remained ocular throughout the follow-up duration (median 11.8 months) in the remaining 155 patients (76.7%). AChR antibody, abnormal repetitive nerve stimulation tests (RNST) and thymoma were more frequently observed in the patients in the OMG-G group than in those in the OMG-R group (p<0.01 in all). In seropositive cases, the titers of AChR antibody were also significantly higher in the OMG-G group than in the OMG-R group (median, 3.8 nM vs. 6.4 nM; p<0.05). Cox proportional hazards regression analyses showed that early oral prednisolone treatment significantly reduced the risk of secondary generalization (HR, 0.24; 95% CI, 0.11-0.56), whereas abnormal AChR antibody (HR, 5.34; 95% CI, 1.60-17.8) and thymoma (HR, 2.32; 95% CI, 1.21-4.45) were predictive of the development of secondary generalization.
Our findings suggest that several factors, including the AChR antibody, thymoma, early corticosteroid treatment, and possibly latent neuromuscular abnormality revealed by RNST, may have an impact on the risk of developing generalized disease in Korean patients presenting with ocular myasthenia.
本研究的目的是获取韩国自身免疫性重症肌无力（MG）患者仅以眼部症状起病并进展为全身型疾病的频率数据，并确定与继发全身型相关的预后因素。
我们进行了一项回顾性多中心调查，对2000年至2005年新诊断为MG的376例成年患者进行了分析。症状出现时为眼肌型MG的患者（n = 202，53.7%）根据预后分为两个亚组：随访期间疾病始终局限于眼部的患者归入OMG-R组，进展为全身型疾病的患者归入OMG-G组。比较了两个亚组的临床特征和实验室检查结果。
202例研究对象中有47例（23.3%）发生继发全身型，大多在症状出现后的前6个月内，而其余155例患者（76.7%）在整个随访期间（中位时间11.8个月）疾病始终局限于眼部。OMG-G组患者中抗乙酰胆碱受体（AChR）抗体、重复神经电刺激试验（RNST）异常及胸腺瘤的发生率高于OMG-R组患者（所有p值均<0.01）。在血清学阳性病例中，OMG-G组AChR抗体滴度也显著高于OMG-R组（中位值，3.8 nM对6.4 nM；p<0.05）。Cox比例风险回归分析显示，早期口服泼尼松治疗显著降低了继发全身型的风险（风险比[HR]，0.24；95%置信区间[CI]，0.11 - 0.56），而AChR抗体异常（HR，5.34；95% CI，1.60 - 17.8）和胸腺瘤（HR，2.32；95% CI，1.21 - 4.45）可预测继发全身型的发生。
我们的研究结果表明，包括AChR抗体、胸腺瘤、早期糖皮质激素治疗以及可能由RNST揭示的潜在神经肌肉异常等多种因素，可能对韩国眼肌型重症肌无力患者发生全身型疾病的风险产生影响。
