【摘要】：重癥肌無力(myasthenia gravis,MG)現(xiàn)多項研究明確為乙酰膽堿受體抗體介導(dǎo)的自身免疫性疾病,主要累及多部位神經(jīng)-肌肉接頭,導(dǎo)致橫紋肌的收縮無力,呈現(xiàn)出特征性特點,波動性和易疲勞性。根據(jù)肌肉的受累范圍,MG分為眼肌型MG(ocular myasthenia gravis,OMG),全身型MG(generalized myasthenia gravis,GMG)兩種臨床亞型。OMG受累肌肉僅限于眼外肌,而GMG廣泛累及全身骨骼肌,預(yù)后較OMG更差,嚴重時因呼吸肌麻痹導(dǎo)致呼吸衰竭,危及患者生命。流行病學(xué)調(diào)查顯示,MG在全球范圍均可發(fā)病,男女均可罹患,但受到地域、種族及性別的影響,在不同地區(qū)或國家MG的臨床表型也呈現(xiàn)出多樣化的特點,我國OMG患者的臨床特點也有待全面分析。有文獻表明,MG患者85%首發(fā)癥狀僅為眼外肌麻痹,即OMG,其中50%左右患者可在半年內(nèi)進展為GMG,80%患者在發(fā)病后1年內(nèi)進展為GMG,90%患者在發(fā)病3年后進展為GMG,導(dǎo)致臨床預(yù)后不良,給患者、家庭及社會都帶來巨大壓力。因此,探尋可預(yù)測OMG向GMG轉(zhuǎn)化的臨床指標(biāo),對于盡早判別及治療高危OMG患者,爭取良好預(yù)后具有積極的實際意義。目的回顧性分析我國OMG患者的臨床特點;根據(jù)起病年齡和神經(jīng)電生理檢查結(jié)果分別進行亞組分析;探尋我國omg向gmg轉(zhuǎn)化的預(yù)測因素。方法查閱在第四軍醫(yī)大學(xué)唐都醫(yī)院神經(jīng)內(nèi)科住院患者,收集2008年6月至2012年6月的確診mg患者的病歷資料,首發(fā)癥狀為單純眼外肌麻痹為的omg患者納入本研究。收集上述患者的基本臨床資料,包括性別、起病年齡、首發(fā)眼部癥狀,特別是新斯的明試驗,低頻重復(fù)電刺激、胸腺影像學(xué)、甲狀腺功能等臨床指標(biāo),綜合分析我國omg臨床特點。2013年6月對所有納入患者進行門診或電話隨訪,對gmg轉(zhuǎn)化情況進行統(tǒng)計,并借助logistic回歸分析探尋omg向gmg轉(zhuǎn)化的預(yù)測因素。結(jié)果本研究共納入來自22個省及直轄市的392例首診為omg患者,其中男性210例,女性182例,男、女比例為1.2:1�；颊咂鸩∧挲g為9個月-81歲,中位起病年齡為20歲,男、女患者在中位起病年齡及各年齡段人數(shù)分布方面無顯著性差異。186例患者(47.4%)在15歲以前起病,是omg的第一個發(fā)病高峰,男、女發(fā)病均等;50-60歲出現(xiàn)患者另一發(fā)病高峰,男性患者居多。366例患者的首發(fā)癥狀表現(xiàn)為或含有上瞼下垂,占所有患者的93.4%,其中單側(cè)受累(269例)明顯多于雙側(cè)受累(97例)。392例患者中有265例(67.6%)以單純上瞼下垂為首發(fā)癥狀,26例(6.6%)為單純復(fù)視,101例(25.8%)為上瞼下垂合并復(fù)視。男、女性患者在不同首發(fā)癥狀中的構(gòu)成比方面無統(tǒng)計學(xué)差異(p=0.439)。125例患者的199只眼球活動受限,最常見的類型是眼球固定,占所有受累眼球的30.7%,單純外展受限(15.6%)和單純上視受限(10.6%)次之。199只活動受限眼球中,65.8%存在外展受限,58.8%存在上視受限,58.3%存在內(nèi)收受限,48.7%存在下視受限。308例患者接受了溴吡斯的明試驗,295例呈陽性(95.8%);男、女患者的陽性率無顯著性差異。面神經(jīng)、腋神經(jīng)、尺神經(jīng)的低頻重復(fù)電刺激的陽性率分別為32.4%、20.2%和2.5%。神經(jīng)電生理結(jié)果顯示男、女患者rns的陽性率方面并無顯著性差異。納入392例患者中169例患者接受了甲狀腺功能檢測,甲狀腺功能的異常者31例,陽性率為(18.3%),甲狀腺功能亢進患者16例,7例為亞臨床甲亢患者,2例為甲狀腺功能減退,6例為亞臨床甲減。280例患者接受胸部ct檢查,58例存在胸腺異常(20.7%),其中36例為胸腺瘤,15例為胸腺增生,7例為胸腺退化異常。共有223例患者完成了最終訪視,其各項臨床指標(biāo)與392例樣本群比較無顯著性差異。亞組分析顯示,成年起病患者的腋神經(jīng)rns陽性率顯著高于青少年起病者(30.8%vs 8.3%,P=0.032);面神經(jīng)RNS陽性患者同時出現(xiàn)腋神經(jīng)RNS陽性的比率顯著高于面神經(jīng)RNS陰性者;同樣,腋神經(jīng)RNS陽性患者同時出現(xiàn)面神經(jīng)RNS陽性的比率顯著高于腋神經(jīng)RNS陰性者。223例患者中有38例轉(zhuǎn)化為GMG,轉(zhuǎn)化率為17.0%。成年起病、面神經(jīng)RNS陽性、腋神經(jīng)RNS陽性患者的累積GMG轉(zhuǎn)化率分別高于青少年起病、面神經(jīng)RNS陰性、腋神經(jīng)RNS陰性患者。與青少年起病患者相比,成年起病患者的GMG轉(zhuǎn)化病程更短(P=0.0139)。多元回歸分析顯示,起病年齡、病程和面神經(jīng)RNS結(jié)果預(yù)測OMG向GMG轉(zhuǎn)化的OR值分別為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)。結(jié)論我國OMG具有獨特的臨床特點:包括男性更常罹患、首發(fā)癥狀以單純上瞼下垂最多見、眼球活動受限以眼球固定最常見、眼動肌肉受累以外直肌最常見、新斯的明試驗陽性率高、低頻RNS以面神經(jīng)陽性率最高、合并甲狀腺功能異常以甲亢最多見、胸腺影像學(xué)異常以胸腺瘤最多見。面神經(jīng)和腋神經(jīng)RNS同時出現(xiàn)陽性的比率較高。起病年齡、病程和面神經(jīng)RNS結(jié)果是OMG向GMG轉(zhuǎn)化的預(yù)測因素,成年起病、病程短、面神經(jīng)RNS陽性患者具有更高的GMG轉(zhuǎn)化風(fēng)險。
[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.
【學(xué)位授予單位】：第四軍醫(yī)大學(xué)
【學(xué)位級別】：碩士
【學(xué)位授予年份】：2014
【分類號】：R746.1

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相關(guān)碩士學(xué)位論文 前10條

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6 馬姍;重癥肌無力患者生活質(zhì)量與睡眠質(zhì)量分析[D];第四軍醫(yī)大學(xué);2015年

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