重症肌无力患者血清抗心肌抗体测定的意义

应用免疫荧光技术，测定重症肌无力（ＭＧ）患者，健康者及其他疾病患者血清心肌抗体。发现２３例伴胸腺瘤ＭＧ患者的抗心肌抗体阳性率为９１．３％，而５４例不伴胸腺瘤ＭＧ患乾的阳性率仅为１８．５％，健康及疾病对照组内无１例阳性。还发现伴心电图异常的ＭＧ患者抗心肌抗体阳性率为７６．５％，而不伴心电图异常者阳性率仅为３０％。结果表明抗心肌抗体的出现与ＭＧ伴胸腺瘤密切相关，且它与心电图异常有一定的关系。此项测定可     

重症肌无力患者血清连接素抗体的检测及其临床意义

目的 探讨连接素（ｔｉｔｉｎ或ｃｏｎｎｅｃｔｉｎ）抗体９＝（ｔｉｔｉｎａｂ）与重症肌无力（ＭＧ）的关系。方法 应用基因工程合成重组重症肌无力胸腺瘤特异性３００００抗原（ＭＧＴ－３０蛋白）,并采用酶联免疫吸附法（ＥＬＩＳＡ）检测１４１例不同胸腺病理类型的ＭＧ患者（ＭＧ组）、２６５名健康对照者（ＮＣ组）和３６例非ＭＧ其他疾病患者（ＮＭＧ组）血清中ｔｉｔｉｎａｂ水平,同时检测ＭＧ患者血清中乙酰胆碱受体抗体（ＡＣｈＲａｂ）、突触前膜受体抗体（ＰｒｓｍＲａｂ）水平。结果 ＭＧ组血清中ｔｉｔｉｎａｂ水平明显高于ＮＣ组和ＮＭＧ组（均Ｐ〈０．０１）;阳性率以ＭＧ伴胸腺瘤（ＭＧＴ）组最高（８３．７％）,ＭＧ伴胸腺萎缩（ＭＧＡ）组其次（４３．２％０,而ＭＧ伴胸腺增生（ＭＧＨ）组及ＭＧ胸腺正常（ＭＧＮ）组患者血清中ｔｉｔｉｎａｂ均为     

伴与不伴注意缺陷多动障碍的抽动秽语综合征患儿动态脑电图的 …

目的 探讨与不伴注意缺陷多动障碍（ＡＤＨＤ）的抽动微语综合征（ＴＳ）患儿间脑电图的差异。方法 对８６例伴与不伴ＡＤＨＤ的ＴＳ患儿进行２４ｈ动态脑电图（ＡＥＥＧ）监测。结果 伴ＡＤＨＤ的ＴＳ组（４０例）ＡＥＥＧ异常率为７５％,单纯ＴＳ组（４６例）异常率为３０％,２组差异有显著性（Ｐ〈０．０５）;ＡＥＥＧ异常的主要表现为慢波异常以及癫痫样波,伴ＡＤＨＤ的ＴＳ组的ＡＥＥＧ局部异常多于广泛异常,且以额叶受     

伴与不伴注意缺陷多动障碍的抽动秽语综合征患儿动态脑电图的对照研究

目的　探讨伴与不伴注意缺陷多动障碍（ＡＤＨＤ） 的抽动秽语综合征（ＴＳ） 患儿间脑电图的差异。方法　对８６ 例伴与不伴ＡＤＨＤ 的ＴＳ患儿进行２４ ｈ 动态脑电图（ＡＥＥＧ） 监测。结果　伴ＡＤＨＤ的ＴＳ组（４０ 例）ＡＥＥＧ异常率为７５％ ,单纯ＴＳ组（４６ 例） 异常率为３０％ ,２ 组差异有显著性（ Ｐ＜０－０５） ;ＡＥＥＧ异常的主要表现为慢波异常以及癫痫样波。伴ＡＤＨＤ的ＴＳ组的ＡＥＥＧ 局部异常多于广泛异常,且以额叶受累多见;而单纯ＴＳ组则广泛异常多于局部异常。结论　伴与不伴ＡＤＨＤ的ＴＳ患儿在脑电生理学上存在异质性     

血清CAE—Ab,nAchR—Ab检测对伴发胸腺瘤的重症肌无力患者？…

目的 探讨柠檬酸提取物抗体（ＣＡＥ－Ａｂ）在烟碱型乙酰胆碱受体抗体（ｎＡｃｈＲ－Ａｂ）对临床早期诊断伴发胸腺瘤的重症肌无力（ＭＧ）患者的价值,与改良Ｏｓｓｅｒｍａｎ分型及病理分型的关系。方法 采用酶免疫吸附法检测２０例伴发胸腺瘤的重症肌无力患者的血清ＣＡＥ－Ａｂ、ｎＡｃｈＲ－Ａｂ。结果 ＣＡＥ－Ａｂ阳性率７５％,ｎＡｃｈＲ－Ａｂ阳性率６０％,大多在Ⅱａ型与Ⅱｂ型患者,上皮细胞为主型４例中Ⅱｂ型３例     

肌球蛋白抗体在重症肌无力病人血清中的表达及其在发病机？…

目的：探讨ｍｙｏｓｉｎ－Ａｂ在ＭＧ发病机制中所起的作用。方法：采用ＥＬＩＳＡ法检测重症肌无力（ＭＧ）组５４例，正常人组９２例及其他疾病对照组２８例血汪肿ｍｙｏｓｉｎ－Ａｂ的表达。结果：Ｍｙｏｓｉｎ－Ａｂ在血清中的正常值Ｐ／Ｎ（病人与正常人ＯＤ值之比）范围：０～２．９８。高于２．９３％则为阳性。Ｍｙｏｓｉｎ－Ａｂ阳性率与肌无力严重程度呈正相关。Ｍｙｏｓｉｎ－Ａｂ效价与ＡＣｈＲ－Ａｂ呈正相关。结论：Ｍ     

抗心磷脂抗体与偏头痛

采用酶联免疫法测定３８例偏头痛患者的ＩｇＧ和ＩｇＭ型抗心磷脂抗体（ＡＣＬ），结果偏头痛组ＡＣＬ水平和ＡＣＬ阳性率与３０例对照组比较均有显著差异（Ｐ＜０．０１），提示偏头痛患者有免疫调节功能异常。     

ABC－ELISA法检测重症肌无力患者三种自身抗体的研究

本文利用ＡＢＣ－ＥＬＩＳＡ法检测了９７例ＭＧ病人血清内三种抗体：ＡｃｈＲａｂ、Ｐｒ－Ｍａｂ、ＣＡＥａｂ。结果发现：（１）全身型ＡｃｈＲａｂ、ｐｒ－Ｍａｂ阳性率明显高于眼肌型（Ｐ＜０．０１）；ＡｃｈＲａｂ与Ｐｒ－ＭａｂＰ／Ｎ值呈线性正相关（ｒ＝０．７９７Ｐ＜０．０１）。（２）合并胸腺异常者ＣＡＥａｂ阳性率为８４．２％，明显高于对照组（Ｐ＜０．０１）；此组病人ＡｃｈＲａｂ与ＣＡＥａｂＰ／Ｎ值呈显著正相关，Ｐｒ－Ｍａｂ与ＣＡＥａｂＰ／Ｎ值呈非常显著正相关。（ｒ＝０．５１２和ｒ＝０．５９８Ｐ＜０．０１）。（３）８例病人作了治疗前后抗体检测。激素治疗后或切除异常胸腺后，抗体滴度多数下降或有转阴趋势。     

重症肌无力患者的心肌酶及心电图分析

目的　探讨重症肌无力 (MG)患者的心脏损害。方法　对 1 0 3例MG患者空腹取血查心肌酶 ,心电图检查。结果　 1 0 3例MG患者中心肌酶异常率 2 6 2 % (2 7/ 1 0 3) ,心电图异常率 2 3 3 % (2 4 / 1 0 3)。伴胸腺瘤的MG患者与不伴胸腺瘤的MG患者之间的心肌酶异常率及心电图异常率均有显著性差异 (P <0 0 5)。眼肌型患者与全身型患者之间的心肌酶异常率及心电图异常率均无显著性差异 (P >0 0 5)。结论　MG患者伴有心脏损害 ,尤其伴胸腺瘤的MG患者。心肌酶及心电图检查可提示MG患者的心脏损害。     

癫痫患者血清中抗脑组织抗体的检测

补体结合试验对１１３例癫痫患者及６０例对照血清中抗脑组织抗体（ａｎｔｉ－ｅｎｃｅｐｈａｌｉｃａｎｔｉｂｏｄｙ，ＡＥＡｂ）进行检测，结果：１．癫痫患者ＡＥＡｂ阳性率为４２．２８％，明显高于对照组阳性率８．３３％（Ｐ＜０．０１）。２．原发性癫痫与继发性癫痫之间ＡＥＡｂ阳性率未见差异；ＡＥＡｂ阳性率与年龄、性别、是否用抗痫药亦无明显关系（Ｐ＞０．０５）。３．ＡＥＡｂ阳性率与癫痫病程长短有关（Ｐ＜０．０５）；与脑电图是否异常、用药效果、发作类型有关（Ｐ＜０．０１）     

The predictive value of the presence of different antibodies and thymus pathology to the clinical outcome in patients with generalized myasthenia gravis

Objectives

To analyze the predictive value of anti-acetylcholine receptor antibodies (anti-AChR Ab) and anti-muscle specific kinase antibodies (anti-MuSK Ab), as well as the thymus pathology to the clinical outcome in patients with generalized myasthenia gravis (MG).

Methods

We analyzed 138 patients with generalized MG, who were thymectomized and assayed for anti-AChR Ab and anti-MuSK Ab.

Results

Anti-AChR Ab were detected in 84% of patients, while anti-MuSK Ab were present in 36% of the AChR Ab negative patients. Severe forms of the disease were more frequent in MuSK Ab positive, compared to the AChR Ab positive and complete seronegative patients. Thymic lymphoid follicular hyperplasia (LFH) was present in 60%, thymoma in 23%, atrophic thymus in 9% and the normal thymus in 8% of patients. LFH was more frequent among women, while thymoma and atrophic thymus were more frequent in men. The younger patients mainly had LFH and normal thymus, while thymoma and atrophic thymus were more frequent in older patients. The mildest clinical presentation was present in patients with normal thymus, while severe forms of the disease were registered in the patients with thymoma. The AChR Ab positive patients had more often LFH and thymoma, while within MuSK Ab positive patients atrophic thymus was most common.

Conclusion

The best disease outcome was observed in patients with normal thymus or LFH with anti-AChR Ab or without both types of antibodies.     

Heart disease in myasthenia gravis

Patients with myasthenia gravis (MG) may develop heart disease. Our data on 108 MG patients were examined to assess the type and frequency of this. 17 of 108 patients (16%) showed signs of heart disease which could be regarded as MG-related. 11 of these, of whom 5 died suddenly, had clinical symptoms, mainly arrhythmias. Signs of heart disease were most frequent in thymoma patients (5 of 10), and all 3 microscopically examined hearts from these patients showed a focal myocarditis. 44 spinal muscular atrophy patients serving as controls showed a 16% frequency of signs of heart disease of unknown etiology. However, only 1 of 44 spinal muscular atrophy patients (2%) had clinical symptoms as compared to 11 of 108 MG patients (10%). Together with the characteristic focal nature of the myocarditis and the microscopic similarities between lesions of heart and skeletal muscle, this indicates that the heart disease is specifically related to MG.     

重症肌无力患者血清抗Titin抗体检测的临床意义

目的探讨重症肌无力(myasthenia gravis,MG)患者血清抗Titin抗体检测的临床意义。方法用重组体MGT-30(提纯的Titin片断)作为抗原,应用ELISA对77例MG患者(包括25例胸腺瘤患者)、58例其他神经系统疾病患者及46例健康对照者进行血清抗Titin抗体检测。结果MG、MG合并胸腺瘤和年龄超过40岁而无胸腺瘤的晚发型MG患者组中抗Titin抗体的阳性率分别为63.6%、80%和70.6%,而其他神经系统疾病组和健康对照者中抗Titin抗体阳性者较少,分别为15.5%、6.5%。MG患者组的阳性率明显高于对照组。结论血清抗Titin抗体检测可作为MG诊断的参考指标,尤其对合并胸腺瘤患者可能更具有临床意义。     

Muscle autoantibodies in subgroups of myasthenia gravis patients

Myasthenia gravis (MG) is caused by autoantibodies to the acetylcholine receptor (AChR), but several other muscle autoantibodies have also been identified in patient sera. We studied muscle autoantibodies against AChR, striated muscle tissue sections (SH), titin, citric acid antigen (CA), and ryanodine receptor (RyR) in sera from 146 consecutive MG patients to evaluate whether a single test or several tests together can predict a thymoma. The MG patients were divided into five subgroups; ocular MG, early-onset MG (< 50 years), late-onset MG (≥ 50 years), MG with thymoma, and AChR antibody negative MG. AChR, SH, titin, CA, and RyR antibodies were detected in 85%, 34%, 34%, 25%, and 14% of the MG patients, respectively. For thymoma MG, AChR, SH, titin, CA, and RyR antibodies were detected in 100%, 75%, 95%, 70%, and 70% respectively. SH, titin, CA, RyR antibodies, and computed tomography of the anterior mediastinum have similar sensitivity for thymoma MG. The specificity of RyR, titin, CA, and SH antibodies for thymoma was 70%, 39%, 38%, and 31%, respectively, which is significantly higher for RyR antibodies than for the others. No single muscle antibody assay can predict a thymoma, and a combination of several antibody assays is preferred, although RyR antibody testing alone showed 70% sensitivity and specificity for thymoma MG. SH and CA antibodies provided only little additional information. Received: 23 September 1999, Received in revised form: 6 December 1999, Accepted: 19 January 2000     

Antibodies against the main immunogenic region of the acetylcholine receptor correlate with disease severity in myasthenia gravis

Objective We developed an assay that detects autoantibodies against the main immunogenic region (MIR) located at the extracellular end of the nicotinic acetylcholine receptor (AChR) α subunit, and investigated its clinical relevance in myasthenia gravis (MG). Methods In this retrospective cohort study, we measured MIR antibody (Ab) titres in sera obtained before treatment and analysed their associations with clinical parameters in 102 MG patients from two neurological centres. MIR Ab titres were determined using a modified competition immunoprecipitation assay in the presence or absence of monoclonal antibody 35. Results 11 of 23 (47.8%) ocular type and 66 of 72 (91.7%) generalised type MG patients were positive for the presence of MIR Abs, defined as a titre >16.8% (3 SDs above the mean for 70 healthy controls). A significantly higher MIR Ab titre (p<0.001) was shown in generalised type (47.9±19.2%) rather than in ocular type MG patients (16.4±8.4%). Bivariate regression analysis using both titre levels of MIR Ab and routine AChR binding Ab as variables revealed MIR Abs to be an exclusive indicator positively associated with disease severity (Myasthenia Gravis Foundation of America classification, p<0.0001; Quantitative MG score, p=0.008), the presence of bulbar symptoms (p<0.0001) and thymoma (p=0.016), and negatively associated with ocular MG (p<0.0001). Conclusions MIR Ab titre levels show much better correlations with factors related to disease severity compared with AChR binding Ab titres. The MIR Ab assay may be useful for predicting MG symptom severity, especially for discriminating between ocular and generalised types of MG.     

Disease severity and outcome in thymoma myasthenia gravis: a long-term observation study

Thymomas occurring in myasthenia gravis (MG) are usually of the cortical subtype and are usually treated by thymectomy. However, the factors that influence MG outcome in thymoma MG patients are not known. In a long-term study, MG severity and treatment was observed in 24 thymoma and 24 non-thymoma MG patients for up to 30 years, and the occurrence of muscle autoantibodies was assayed. The rate of complete stable remission was low and did not differ between the two groups. There was no significant difference in MG severity between thymoma and non-thymoma MG patients at any time during the study. Titin and ryanodine receptor (RyR) antibody occurrence was significantly higher in thymoma MG patients. Four thymoma (all titin and RyR antibody positive) and two non-thymoma (both titin and one RyR antibody positive) MG patients died from MG-related respiratory insufficiency. Seventy percent of thymoma and 75% of non-thymoma MG patients were treated with immunosuppressive drugs. The number of patients who received plasmapheresis did not differ in those who were treated because of acute MG deterioration, irrespective of planned surgery.This study shows equal MG severity and outcome in thymoma and non-thymoma MG, but the presence of RyR antibodies in thymoma MG and titin/RyR in non-thymoma MG indicates a less favorable prognosis.     

High frequency of allergic conjunctivitis in myasthenia gravis without thymoma

OBJECTIVES: To investigate the frequency of allergic disorders in myasthenia gravis (MG) patients and characterize the features of MG associated with allergic disorders. METHODS: Frequencies of past and present common allergic disorders in 160 MG patients who visited the Department of Neurology, Kyushu University Hospital from April 2000 to July 2003 and in 81 neurological normal controls were studied. RESULTS: Among various allergic disorders, the frequency of allergic conjunctivitis (AC) was significantly higher in MG patients (39/160, 24.4%, p(corr)=0.0112), especially with MG without thymoma (36/123, 29.3%, p(corr)=0.0016), in comparison to the controls (6/81, 7.4%). MG patients with AC showed a significantly higher rate of seronegative MG (43.6% vs. 17.4%, p=0.008) and a higher tendency of ocular MG (43.6% vs. 28.1%, p=0.071). Moreover, MG with AC had significantly lower anti-acetylcholine receptor antibody titers (median 6.8 nmol/l vs. median 23.6 nmol/l, p=0.0359) as well as a lower rate of coexisting thymoma (7.7% vs. 17.4%, p=0.016). The incidence of myasthenic crisis was also lower in MG with AC than without AC, yet the difference was not significant (7.7% vs. 15.7%). CONCLUSION: There was a significant association of AC with MG especially for ocular or seronegative MG in cases without thymoma.     

重症肌无力患者血清干扰素-α抗体的检测意义

目的研究重症肌无力（MG）患者外周血中干扰素α抗体（IFN-αAb）的含量,并探讨其与MG的关系。方法采用ELISA法测定60例MG患者、20例正常对照组（NC）及20例非MG其他神经系统疾病患者（OND）血清中IFN-αAb。结果 发现伴胸腺瘤的重症肌无力患者（MGT）血清中IFN-αAb阳性率为75%,明显高于不伴胸腺瘤的重症肌无力患者（NTMG,11.5%）及对照组（P〈0.05）;晚发型MG者IFN-αAb阳性率为29.41%,明显高于早发型MG患者7.69%（P〈0.05）。结论 伴胸腺瘤的MG患者及晚发型MG患者外周血中IFN-αAb表达增高。     

Detection of antibodies to citric acid extract of skeletal muscle (CAE-ab) in the sera of patients with myasthenia gravis using indirect hemagglutination

In this paper are described the method and results for the demonstration of the antibodies to citric acid extract of skeletal muscle (CAE-ab) in the sera from myasthenia gravis (MG) patients with or without thymoma, in patients with other diseases and in healthy controls by an indirect hemagglutination assay (IHA), were described. The CAE-ab titers were positive in 15 (71.43%) of the 21 MG patients with thymoma, the antibody titers ranging from 1:16 to 1:512. In the 35 MG patients without thymoma, as well as in the 32 healthy controls and 22 patients with other diseases, the antibody titers were all negative A statistically significant difference was found between the CAE-ab titers in the group of MG patients with thymoma and those in the other three groups. It was considered that IHA for the purpose of demonstrating CAE-ab could serve as a supplementary diagnostic method for early detection of thymoma in MG patients at an early stage.     

Acetylcholine receptor antibodies in myasthenia gravis

A multivariate statistical analysis of levels of serum acetylcholine receptor antibody (AChR Ab) obtained from 197 patients with various clinical forms of myasthenia gravis (MG) was performed. Elevated AChR Ab levels are specific for MG, but normal AChR Ab levels do not rule out MG. Patients in remission or with purely ocular MG had the lowest incidence of elevation of serum AChR Ab levels, while patients with generalized, severe MG, particularly in the presence of thymoma, tended to have the greatest antibody elevations. Corticosteroids depressed AChR Ab levels, but thymectomy did not exert a consistent effect on antibody levels within a 24- to 30-month postoperative period. The relatively low 55% positivity of antibody elevations in all 197 patients probably reflects the use of heterologous (rat) AChR.