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Single-fiber Electromyography in the Extensor Digitorum Communis for the Predictive Prognosis of ocular myasthenia gravis： A Retrospective Study of 102 Cases

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Chinese Medical Journal 2015年第20期128卷 2783-2786页

作者： Yu-Zhou Guan Li-Ying Cui Ming-Sheng Liu Jing-Wen Niu Department of Neurology Peking Union Medical College Hospital Chinese Academy of Medical Sciences Beijing 100730 China Neuroscience Center Chinese Academy of Medical Sciences Beijing 100730 China

Background： Single-fiber electromyography （SFEMG） abnormality in the extensor digitorum communis （EDC） was reported in ocular myasthenia gravis （OMG）, which indicated subclinical involvement beyond extraocular muscles in OMG patients. The relationship between the abnormal findings of SFEMG in EDC and the probability for OMG to develop generalized myasthenia gravis （GMG） is unknown, This retrospective study aimed to determine the predictive value of abnormality of SFEMG in EDC of OMG patients. Methods： One-hundred and two OMG patients underwent standard clinical diagnosis process and SFEMG test in EDC muscle when diagnosed and were clinically followed up for 5 years. The SFEMG data were compared between different clinical groups according to thymus status, onset age, and different outcome of OMG developing. Chances of progressing to GMG were compared between two different groups according to SFEMG and repetitive nerve stimulation （RNS） results, acetylcholine receptor antibody （AchRAb） titer, thymus status, and onset age. Results： Abnormal SFEMG results were observed in 84 （82.4%） patients. The mean jitter, percentage of jitter 〉55 Its （%）, and blocking were higher in OMG patients than in healthy volunteers. There were no statistical differences in jitter analysis between thymoma group and non-thymoma group （P = 0.65）, or between the later OMG group and the later GMG group （P = 0.31）, including mean jitter, percentage of jitter 〉55 Its （%）, and blocking. Elderly group （〉45 years old） had a higher mean jitter than younger group （t = 2.235, P = 0.028）. Total 55 OMG developed GMG, including 47 in abnormal SFEMG group while 8 in normal SFEMG group. There was no statistical difference in the conversion rates between the two groups （x^2 = 0.790, P = 0.140）. RNS abnormality, AchRab titer, or onset age had no correlation with OMG prognosis （P = 0.150, 0.070, 0.120, respectively） while thymoma did （x^2 = 0.510, P = 0.020）. Conclusion： SFEMG test in the EDC showed high abnormal

关键词： Extensor Digitorum Communis Generalized myasthenia gravis ocular myasthenia gravis Prognosis Single-fiberElectromyography

Clinical Features and Prognosis of ocular myasthenia gravis Patients with Different Phenotypes

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Chinese Medical Journal 2015年第19期128卷 2682-2684页

作者： Li-Li Wang Yun Zhang Mao-Lin He Department of Neurology Beijing Shijitan HospitalCapital Medical UniversityBeijing 100038China

INTRODUCTIONmyasthenia gravis （MG） is an autoimmune neuromuscular disorder caused by anti-acetylcholine receptor antibodies （AChR-Abs） or other *** symptoms were the first presentations in 40-50％ of MG *** and diplopia were the most common symptoms of ocular MG （OMG）.Limited studies demonstrated that patients presented with initial symptoms of only ptosis in 47％,only diplopia in 14％ and both ptosis and diplopia in 39％ of OMG.[1] Variations of diplopia or ptosis did not significantly affect the physical or mental status of OMG.

关键词： Clinical Features Diplopia ocular myasthenia gravis Prognosis Ptosis

Sixty cases of ocular myasthenia gravis treated with acupuncture and moxibustion at extra-meridian and collateral

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World Journal of Acupuncture-Moxibustion 2012年第4期22卷 56-59页

作者：陈金亮潘朝刘艳君 LIU Zhao 河北医科大学附属以岭医院

Objective To explore effective therapeutic method for the treatment of ocular myasthenia gravis. Methods Sixty cases of ocular myasthenia gravis were selected. The acupuncture was applied at Dàzhuī （大椎 GV 14）, Bǎihuì （百会 GV 20）, Shàngxīng （上星 GV 23） of Governor Vessel, and its crossing points with Yinqiao and Yangqiao Meridian, such as Jīngmíng （睛明 BL 1）, shēnmài （申脉 BL 62）, Dìcāng （地仓 ST 4）, Jūliáo （巨髎 ST 3）, Chéngqì （承泣 ST 1 ）, etc. Besides, ginger-partition moxibustion was applied at Yángbái （阳白 GB 14） and Sìbái （四白 ST 2）. The plum-blossom needle was applied at upper limb distribution of lung channel of hand taiyin, heart channel of hand shaoyin and pericardium channel of hand jueyin. The patients’ clinical symptoms, potential degradation percentages of repetitive nerve stimulation （RNS） in low frequency were observed. Results 8 cases were clinically cured, 20 cases were basically cured, 12 cases were remarkably effective, 15 cases were effective and 5 cases were failed. The total effective rate was 91.7%. Compared with before the treatment, the potential degradation percentages of repetitive nerve stimulation in low frequency were lowered [（19.44±6.28）% vs （8.78±3.12）%, （P0.05）]. Conclusion Acupuncture and moxibustion at extra-meridian and collateral for ocular myasthenia gravis could achieve positive efficacy.

关键词： ocular myasthenia gravis acupuncture therapy extra-meridian and collateral plum-blossom needle prick

CD4+CD25+调节性T细胞对成人眼肌型重症肌无力临床转归的影响

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CD4+CD25+调节性T细胞对成人眼肌型重症肌无力临床转归的影响

作者：吴元波安徽医科大学

学位级别：硕士

目的重症肌无力（MG）是一种自身免疫性疾病,致病部位在神经肌肉接头,可以选择性的造成肌肉疲劳的现象,对大多数的病例而言,免疫攻击的靶点是骨骼肌的乙酰胆碱受体。约一半的MG患者在初始症状为眼睑下垂,复视,很多人会从单纯的眼肌型重... 详细信息

目的重症肌无力（MG）是一种自身免疫性疾病,致病部位在神经肌肉接头,可以选择性的造成肌肉疲劳的现象,对大多数的病例而言,免疫攻击的靶点是骨骼肌的乙酰胆碱受体。约一半的MG患者在初始症状为眼睑下垂,复视,很多人会从单纯的眼肌型重症肌无力（OMG）向全身型重症肌无力（GMG）进展,患者寄希望于非药物治疗和胆碱酯酶抑制剂,但是最终仍然要口服皮质类固醇或者是免疫抑制剂来控制病情的发展,OMG的发病机制到现在也是不明确。调节性T细胞在免疫反应的控制和平衡,免疫耐受的机制上保持了重要的作用。这可能是影响OMG重要的影响因子。本研究探讨了影响成人OMG的临床转归的因素。方法选择2004年1月-2009年3月在我院门诊以及病房诊断明确的眼肌型重症无力患者共148例,初诊时间作为起点,OMG进展为GMG的时间作为终点,进展组74例患者,67个患者在随访结束的时候仍旧是眼肌型重症肌无力。年龄14-72岁,平均（38.81±14.62）岁;随访期限内,7个患者失访,本研究利用流式细胞仪测患者外周血CD4CD25调节性T细胞占CD4T细胞的百分比。人口学特征（性别,年龄）,有无合并胸腺瘤或者是胸腺增生和胸腺不完全退化,加用类固醇激素和或者是免疫抑制剂,CD4CD25调节性T细胞占CD4细胞比例作为自变量,重症肌无力眼肌型转变为全身型的时间作为因变量进行Cox回归分析。结果Cox回归分析结果提示:免疫抑制剂[95% CI （0.25-0.96） P=0.038]和CD4CD25细胞占CD4细胞比例[95% CI （0.73-0.91）,p<0.01]这两个变量, CD4 CD25调节性T细胞占CD4细胞比例越大,在治疗方案中使用或者加用免疫抑制剂,重症肌无力眼肌型进展为全身型的风险越小,这两个变量是眼肌型重症肌无力临床转化的保护性因素。CD4CD25细胞占CD4细胞比例和眼肌型重症肌无力进展为全身型的时间的偏相关系数0.567（p<0.05）,两者相关关系较显著。结论: CD4CD25调节性T细胞的缺陷是MG发病机制的重要方面,在OMG中的患者中,调节性T细胞有强大的免疫对抗性质,在部分OMG患者存在数量偏少或者是功能缺陷的问题,这可能是引起OMG进展的原因。另外,免疫抑制剂的应用可能是防止OMG向GMG进展的保护性因素。

关键词： CD4+ CD25+ Tregulatory ocular myasthenia gravis Outcome Immunosuppressan