我国OMG临床特点及其向GMG转化的预测因素探讨

发布时间：2018-07-28 17:22

【摘要】：重症肌无力(myasthenia gravis,MG)现多项研究明确为乙酰胆碱受体抗体介导的自身免疫性疾病,主要累及多部位神经-肌肉接头,导致横纹肌的收缩无力,呈现出特征性特点,波动性和易疲劳性。根据肌肉的受累范围,MG分为眼肌型MG(ocular myasthenia gravis,OMG),全身型MG(generalized myasthenia gravis,GMG)两种临床亚型。OMG受累肌肉仅限于眼外肌,而GMG广泛累及全身骨骼肌,预后较OMG更差,严重时因呼吸肌麻痹导致呼吸衰竭,危及患者生命。流行病学调查显示,MG在全球范围均可发病,男女均可罹患,但受到地域、种族及性别的影响,在不同地区或国家MG的临床表型也呈现出多样化的特点,我国OMG患者的临床特点也有待全面分析。有文献表明,MG患者85%首发症状仅为眼外肌麻痹,即OMG,其中50%左右患者可在半年内进展为GMG,80%患者在发病后1年内进展为GMG,90%患者在发病3年后进展为GMG,导致临床预后不良,给患者、家庭及社会都带来巨大压力。因此,探寻可预测OMG向GMG转化的临床指标,对于尽早判别及治疗高危OMG患者,争取良好预后具有积极的实际意义。目的回顾性分析我国OMG患者的临床特点;根据起病年龄和神经电生理检查结果分别进行亚组分析;探寻我国omg向gmg转化的预测因素。方法查阅在第四军医大学唐都医院神经内科住院患者,收集2008年6月至2012年6月的确诊mg患者的病历资料,首发症状为单纯眼外肌麻痹为的omg患者纳入本研究。收集上述患者的基本临床资料,包括性别、起病年龄、首发眼部症状,特别是新斯的明试验,低频重复电刺激、胸腺影像学、甲状腺功能等临床指标,综合分析我国omg临床特点。2013年6月对所有纳入患者进行门诊或电话随访,对gmg转化情况进行统计,并借助logistic回归分析探寻omg向gmg转化的预测因素。结果本研究共纳入来自22个省及直辖市的392例首诊为omg患者,其中男性210例,女性182例,男、女比例为1.2:1。患者起病年龄为9个月-81岁,中位起病年龄为20岁,男、女患者在中位起病年龄及各年龄段人数分布方面无显著性差异。186例患者(47.4%)在15岁以前起病,是omg的第一个发病高峰,男、女发病均等;50-60岁出现患者另一发病高峰,男性患者居多。366例患者的首发症状表现为或含有上睑下垂,占所有患者的93.4%,其中单侧受累(269例)明显多于双侧受累(97例)。392例患者中有265例(67.6%)以单纯上睑下垂为首发症状,26例(6.6%)为单纯复视,101例(25.8%)为上睑下垂合并复视。男、女性患者在不同首发症状中的构成比方面无统计学差异(p=0.439)。125例患者的199只眼球活动受限,最常见的类型是眼球固定,占所有受累眼球的30.7%,单纯外展受限(15.6%)和单纯上视受限(10.6%)次之。199只活动受限眼球中,65.8%存在外展受限,58.8%存在上视受限,58.3%存在内收受限,48.7%存在下视受限。308例患者接受了溴吡斯的明试验,295例呈阳性(95.8%);男、女患者的阳性率无显著性差异。面神经、腋神经、尺神经的低频重复电刺激的阳性率分别为32.4%、20.2%和2.5%。神经电生理结果显示男、女患者rns的阳性率方面并无显著性差异。纳入392例患者中169例患者接受了甲状腺功能检测,甲状腺功能的异常者31例,阳性率为(18.3%),甲状腺功能亢进患者16例,7例为亚临床甲亢患者,2例为甲状腺功能减退,6例为亚临床甲减。280例患者接受胸部ct检查,58例存在胸腺异常(20.7%),其中36例为胸腺瘤,15例为胸腺增生,7例为胸腺退化异常。共有223例患者完成了最终访视,其各项临床指标与392例样本群比较无显著性差异。亚组分析显示,成年起病患者的腋神经rns阳性率显著高于青少年起病者(30.8%vs 8.3%,P=0.032);面神经RNS阳性患者同时出现腋神经RNS阳性的比率显著高于面神经RNS阴性者;同样,腋神经RNS阳性患者同时出现面神经RNS阳性的比率显著高于腋神经RNS阴性者。223例患者中有38例转化为GMG,转化率为17.0%。成年起病、面神经RNS阳性、腋神经RNS阳性患者的累积GMG转化率分别高于青少年起病、面神经RNS阴性、腋神经RNS阴性患者。与青少年起病患者相比,成年起病患者的GMG转化病程更短(P=0.0139)。多元回归分析显示,起病年龄、病程和面神经RNS结果预测OMG向GMG转化的OR值分别为1.023(95%CI 1.006-1.041,P=0.007)、0.603(95%CI 0.365-0.850,P=0.019)和2.826(95%CI 1.045-5.460,P=0.038)。结论我国OMG具有独特的临床特点:包括男性更常罹患、首发症状以单纯上睑下垂最多见、眼球活动受限以眼球固定最常见、眼动肌肉受累以外直肌最常见、新斯的明试验阳性率高、低频RNS以面神经阳性率最高、合并甲状腺功能异常以甲亢最多见、胸腺影像学异常以胸腺瘤最多见。面神经和腋神经RNS同时出现阳性的比率较高。起病年龄、病程和面神经RNS结果是OMG向GMG转化的预测因素,成年起病、病程短、面神经RNS阳性患者具有更高的GMG转化风险。
[Abstract]:Myasthenia gravis (MG) is a kind of autoimmune disease mediated by acetylcholine receptor antibody. It mainly involves multiple parts of the nerve muscle joint, which leads to the weakness of the contractile muscle of the rhabdomyus muscle. It is characterized by characteristics, volatility and fatigue. According to the range of muscle involvement, MG is divided into ocular myasth (ocular myasth). Enia gravis, OMG), systemic MG (generalized myasthenia gravis, GMG) two clinical subtypes of.OMG affected muscles are limited to the extraocular muscles, and GMG is widely involved in the skeletal muscle, and the prognosis is worse than that of OMG. It is serious when respiratory muscle paralysis causes respiratory failure and endanger the patient's life. Epidemiological investigation shows that MG is all around the world, both men and women. But affected by region, race and sex, the clinical phenotypes of MG in different regions and countries are also diverse, and the clinical characteristics of OMG patients in China need to be analyzed. The literature shows that the first symptom of MG patients is only extraocular muscle paralysis, that is, OMG, and about 50% of them can progress to GMG and 80% in half a year. 1 years after the onset of the onset of GMG, 90% patients in 3 years after the onset of GMG, leading to poor clinical prognosis, to the patients, family and society to bring great pressure. Therefore, to explore the predictability of the OMG to GMG transformation of the clinical indicators, for the early discrimination and treatment of high-risk OMG patients, for good prognosis is of positive practical significance. Analysis of the clinical characteristics of OMG patients in China; subgroup analysis according to the onset age and the results of electrophysiological examination; explore the predictive factors for the transformation of OMG into GMG in our country. Methods to consult the hospitalized patients in the Department of Neurology of Tangdu Hospital of The Fourth Military Medical University and collect the medical records of the confirmed MG patients from June 2008 to June 2012 and the first symptom. OMG patients with simple extraocular muscle paralysis were included in this study. The basic clinical data of these patients were collected, including sex, onset age, first eye symptoms, especially neostigmine test, low frequency repetitive electrical stimulation, thymic imaging, thyroid function and other clinical indicators, comprehensive analysis of our country's OMG clinical characteristics in June of all nano.2013 The patients were followed up by outpatient or telephone follow-up, and the GMG transformation was statistically analyzed, and the predictive factors for the conversion of OMG to GMG were explored with the help of logistic regression analysis. Results this study included 392 cases of OMG patients from 22 provinces and municipalities directly under the central government, including 210 men, 182 females and 9 months -81 in the male and female proportion of 1.2:1. patients. The age of onset was 20 years old. There was no significant difference in the age of the onset of the onset of the middle age and the number of all age groups in male and female patients..186 patients (47.4%) were onset before 15 years of age. It was the first peak of the onset of OMG, men and women were equal; the onset of another onset at 50-60 years of age, and the first symptoms of more.366 patients in male patients. For or containing ptosis of the upper eyelid, 93.4% of all patients, of which unilateral involvement (269 cases) was significantly more than bilateral involvement (97 cases) (97 cases), 265 cases (67.6%) were the first symptom of ptosis, 26 cases (6.6%) were simple diplopia, 101 (25.8%) was the combination of upper eyelid drooping and diplopia. Male, female patients were formed in different initial symptoms. There were no statistical differences (p=0.439) in 199.125 patients with limited eye movement, the most common type was eyeball fixation, 30.7% of all affected eyeballs, only abduction Limited (15.6%) and.199 only limited (10.6%) only limited activity in the eyeball, 65.8% had abduction limited, 58.8% had upper limit, 58.3% had adduction limitation, 48.7 The positive rate of 295 patients with.308 was positive (95.8%), and there was no significant difference in the positive rate between men and women. The positive rate of low frequency repetitive electrical stimulation of the facial nerve, the axillary nerve and the ulnar nerve was 32.4%, and the 20.2% and 2.5%. electrophysiological results showed that the positive rate of RNs was not significant in women. Of the 392 patients, 169 patients received thyroid function testing, 31 cases of thyroid dysfunction, 31 cases of positive thyroid function, 16 cases of hyperthyroidism, 7 subclinical hyperthyroidism, 2 hypothyroidism, 6 subclinical hypothyroidism.280 patients receiving chest CT examination, 58 cases of thymic abnormality (20.7 There were 36 cases of thymoma, 15 cases of thymus hyperplasia and 7 cases of thymic degeneration. A total of 223 patients completed the final visit, and there were no significant differences in the clinical indicators and 392 sample groups. The subgroup analysis showed that the positive rate of RNs in the axillary nerve in the adult onset patients was significantly higher than that of the young patients (30.8%vs 8.3%, P=0.032); The ratio of RNS positive of axillary nerve in RNS positive patients was significantly higher than that of RNS negative of facial nerve. Similarly, the ratio of RNS positive of facial nerve in RNS positive patients of axillary nerve was significantly higher than that of RNS negative axillary nerve, 38 cases were converted to GMG, the conversion rate was 17.0%. adult onset, facial nerve RNS positive, axillary nerve RNS positive. The cumulative GMG conversion rate of the patients was higher than that of adolescent onset, RNS negative of facial nerve, and negative axillary RNS negative patients. Compared with young patients, the course of GMG transformation in adult onset patients was shorter (P=0.0139). Multivariate regression analysis showed that the age of onset, course of disease and RNS results of facial nerve predicted that the OR value of OMG to GMG was 1.023 (95%CI 1). .006-1.041, P=0.007), 0.603 (95%CI 0.365-0.850, P=0.019) and 2.826 (95%CI 1.045-5.460, P=0.038). Conclusion Chinese OMG has unique clinical characteristics: the most common symptoms include men, the most common symptoms are simple upper eyelid drooping, the most common eyeball fixation is eyeball fixation, and the most common external rectus muscles involved in the eye movement muscle involvement are the new test. The positive rate of the RNS was highest, the positive rate of the facial nerve was the highest at low frequency. The most common thyroid dysfunction was hyperthyroidism, the most common thymoma was thymoma. The positive rate of the RNS in the facial nerve and the axillary nerve was higher. The onset age, the course of disease and the RNS result of the facial nerve were the predictors of the transformation of the OMG to GMG, and the adult onset and the course of the disease were short. Patients with RNS positive facial nerve have a higher risk of GMG transformation.
【学位授予单位】：第四军医大学
【学位级别】：硕士
【学位授予年份】：2014
【分类号】：R746.1

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