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。     

重症肌无力患者血清Titin抗体和AchR抗体检测的意义

目的 探讨重症肌无力(MG)患者血清Titin抗体及乙酰胆碱受体(AchR)抗体的检测意义.方法 采用酶联免疫吸附试验(ELISA)对81例MG患者和80例对照组成员进行Titin和AchR抗体的检测.结果 Titin抗体对MG具有特异性,它在合并胸腺瘤的MG(MGT)、晚发型MG患者中有较高的阳性率(分别为80%、69.4%)和抗体水平,明显高于早发型MG患者的阳性率(25%)和抗体水平,差异均有统计学意义(P<0.05).而早发型MG患者中AchR抗体阳性率比晚发型MG患者明显增高.并且早发型MG患者的AchR抗体水平也明显高于晚发型MG和MGT患者,差异均有统计学意义(P<0.05).结论 两种抗体检测为MG诊断和病因学研究提供了重要证据,联合检测可以提高MG诊断的灵敏度.     

早、晚发型重症肌无力患者临床特点对比研究

目的研究早、晚发型重症肌无力(MG)患者的临床特点。方法回顾性研究同期住院及门诊的191例MG患者资料,比较早、晚发型MG组患者的构成情况、首发症状、临床分型、伴随疾病、辅助检查、治疗及疗效等临床特点。结果 MG发病以晚发型人群为主,占63.35%。早发型MG组中,男∶女=1∶1.80;晚发型组中,男:女=1:0.92。两组患者首发症状及osserman分型构成比无统计学差异(P0.05),都以Ⅱ型居多,约占早发型58.57%,晚发型57.85%,但晚发型组Ⅱb型较早发型组比例高。晚发型伴随高血压、2型糖尿病、高脂血症比例高(分别42.98%和8.57%,23.97%和2.86%,13.22%和0%;P0.05),而胸腺瘤、甲状腺疾病、其他免疫疾病、肿瘤及副肿瘤综合征在两组患者中差异无统计学意义(P0.05)。早发型MG患者伴胸腺增生构成比例高,晚发型伴胸腺瘤构成比例高,男性患者胸腺瘤占比例高,女性患者胸腺增生占比例高,差异均有统计学意义(P0.05)。女性MG患者伴随甲状腺疾病比例高(P0.05)。两组患者行血清Titin-Ab检查,早发型患者阳性率较晚发型高(P0.05),余血清抗体检测、电生理检查和新斯的明试验比较,差异均无统计学意义(P0.05)。两组患者治疗有效率差异无统计学意义(P0.05),但晚发型有效率较早发型低。结论早、晚发型MG患者在性别构成、首发症状、伴随疾病、辅助检查及预后有所不同,在诊断及治疗时需要注意。     

重症肌无力患者血清titin抗体和乙酰胆碱受体抗体与疾病临床特征的关系

目的探讨重症肌无力(MG)患者血清titin抗体及乙酰胆碱受体(AChR)抗体与疾病临床特征的关系。方法采用酶联免疫吸附试验对90例MG患者和30例对照组成员进行titin抗体和AChR抗体的检测并进行比较。结果 MG患者AChR抗体、Titin抗体阳性率(76.7%,54.5%)明显高于对照组(10%,6.7%)(χ2=41.667,P=0.000;χ2=21.017,P=0.000)。titin抗体与AChR抗体检测MG的灵敏度分别为54.5%和76.7%,两种抗体并联试验灵敏度为89.4%。titin抗体在伴胸腺病变及晚发型MG患者中有较高阳性率,分别为80.0%和72.5%,明显高于非胸腺瘤组(47.1%,χ2=6.771,P=0.009)和早发型MG患者(40%,χ2=9.46,P=0.002)。全身型MG患者titin抗体与AChR抗体阳性率(69.2%,90.4%)显著高于眼肌型MG(34.2%,χ2=10.856,P=0.001;57.9%,χ2=12.956,P≤0.000)。甲亢组与非甲亢组MG患者titin抗体(33.3%,56.8%)与AChR抗体阳性率(55.6%,79%)差异性均无统计学意义(χ2=1.797,P=0.19;χ2=2.491,P=0.063)。结论 MG患者病情越重,titin抗体与AChR抗体阳性率越高。Titin抗体及AChR抗体能作为诊断MG的指标,联合检测能提高MG诊断的灵敏。然而MG患者血清titin抗体与AChR抗体并无一致性关系。     

重症肌无力患者血清Ryanodine受体抗体检测及意义

目的探讨Ryanodine受体（RyR）抗体在重症肌无力（myasthenia gravis，MG）诊断中的临床意义。方法采用ELISA法检测89例MG患者、66例其他神经系统疾病患者和66名正常对照者血清RyR抗体水平。结果MG组血清RyR抗体阳性率显著高于其他神经系统疾病组和正常对照组（P〈0．05），其敏感性和特异性分别为55．0％和91．7％。晚发型MG患者血清RyR抗体阳性率（74．4％）明显高于早发型MG（37．0％，P〈0．05）。MG合并胸腺瘤（MGT）和未合并胸腺瘤（nMGT）患者血清RyR抗体阳性率差异无统计学意义（P〉0．05）。将MG患者根据Osserman评分进行分型，各型血清RyR抗体阳性率及其吸光度值大小与病情严重程度无相关性（P〉0．05）。结论RyR抗体多见于晚发型MG，对诊断MG具有较高的敏感性和特异性。     

重症肌无力患者血清四种抗体的检测及意义

目的探讨乙酰胆碱受体(AChR)抗体、乙酰胆碱酯酶(ACHE)抗体、Titin抗体、Ryanodine受体(RyR)抗体在重症肌无力(myasthenia gravis,MG)诊断中的临床意义。方法采用ELISA法检测89例MG患者、66例其他神经系统疾病患者和66名健康对照者上述血清4种抗体水平。结果 MG患者AChR抗体、AChE抗体、Titin抗体和RyR抗体的阳性率分别为53.9%、20.2%、64.0%和55.0%,明显高于对照组(P0.05)。4种抗体联合检测,其敏感度和特异度分别为94.4%和84.0%。AChE抗体多见于AChR抗体阴性且对新斯的明试验反应差的MG患者。Titin抗体在MG合并胸腺瘤(MGT)患者中的阳性率高于未合并胸腺瘤的MG(nMGT)患者(P0.05),其对MGT诊断的敏感度和特异度分别为82.2%和52.5%。晚发型MG患者血清Titin抗体和RyR抗体阳性率明显高于早发型MG(P0.05)。将MG患者根据Osserman评分进行分型,Titin抗体阳性率与病情严重程度有关(P0.05)。结论 AChR抗体、AChE抗体、Titin抗体和RyR抗体联合检测对诊断MG具有较高的敏感度和特异度。Titin抗体结合胸腺CT可能有助于提高MGT诊断的敏感度和特异度,Titin抗体多见于病情较重的患者。     

晚发型重症肌无力的研究进展(综述)

重症肌无力 ( MG)在老年人口中发病率上升。流行病学资料支持以 5 0岁来区分早发 MG与晚发 MG。早发 MG与晚发 MG主要的免疫学差异是存在抗连接素的自身抗体 ,可在约 5 0 %的晚发 MG患者的血清中探测到。晚发 MG的治疗需针对患者的年龄和其特殊的免疫学表现两方面。     

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。     

全身型重症肌无力的临床和电生理特点分析

目的 探讨不同亚型全身型重症肌无力(Myasthenia gravis,MG)临床和电生理特点之间的区别,并分析电生理结果与疾病严重程度的相关性。方法 回顾性分析2016年7月至2020年7月在香港大学深圳医院神经内科就诊或住院的101例全身型MG患者的临床和电生理资料,按照其临床特点,分为早发乙酰胆碱受体(Acetylcholine receptor,AchR)抗体阳性全身型MG组(52例)、晚发AchR抗体阳性全身型MG组(23例)、胸腺瘤型MG组(22例)和肌肉特异性受体酪氨酸激酶(Muscle-specific receptor tyrosine kinase,MuSK)抗体阳性型MG组(4例),比较不同亚组MG患者的人口学特点、重症肌无力评分(Quantitative Myasthenia Gravis score,QMGs)、美国重症肌无力协会(MG Foundation America,MGFA)临床分级、重复神经电刺激(Repetitive nerve stimulation,RNS)结果,分析RNS结果与QMGs之间的相关性。结果 非MuSK抗体阳性全身型MG中,早发AchR抗体阳性全身型MG女性患者所占比率最高(71. 2%vs 65. 2%vs 36. 4%,P 0. 05),胸腺瘤型MG呼吸肌/球部肌肉受累为主患者所占比率最高(28. 8%vs 43. 5%vs 63. 6%,P 0. 05),早发AchR抗体阳性全身型MG被检肌肉RNS均为阳性患者所占比率最高(44. 2%vs 17. 4%vs 22. 7%,P 0. 05),QMGs与RNS最大波幅下降比率、RNS总波幅下降比率呈明显正相关关系(P 0. 01); MuSK抗体阳性全身型MG,其中重度受累患者所占比率、呼吸肌/球部肌肉受累为主患者所占比率均较高(均为75%),而被检肌肉RNS均为阳性患者所占比率较低(0%)。结论 不同亚型全身型MG的人群分布、性别占比、主要受累肌肉、受累肌肉受累程度及分布范围均具有显著的差别,这在全身型MG的分型诊断方面具有很大的参考价值;其RNS结果与疾病严重程度具有高度相关性,提示RNS在全身型MG的病情评估方面具有重要临床价值。     

重症肌无力患者血清学标志物检测及临床意义

目的探讨血清抗乙酰胆碱受体(ACh R)抗体、抗连接素(Titin)抗体和抗Ryanodine受体(Ry R)抗体对重症肌无力的诊断价值。方法采用酶联免疫吸附试验分别检测182例重症肌无力患者(MG组)、105例其他神经系统疾病患者(OND组)和62例正常对照者(对照组)血清抗ACh R抗体、抗Titin抗体和抗Ry R抗体水平。结果重症肌无力患者血清抗ACh R抗体、抗Titin抗体和抗Ry R抗体阳性率分别为68.13%(124/182)、64.29%(117/182)和67.03%(122/182),高于OND组(均P=0.000)和对照组(均P=0.000);3种血清学标志物同时阳性,诊断重症肌无力的灵敏度为41.21%,特异度达99.40%。其中,早发型与晚发型重症肌无力亚组血清抗ACh R抗体、抗Titin抗体和抗Ry R抗体阳性率差异无统计学意义(均P0.05);伴胸腺瘤的重症肌无力亚组仅血清抗ACh R抗体阳性率高于不伴胸腺瘤的重症肌无力亚组(P=0.004),而抗Titin抗体和抗Ry R抗体阳性率差异无统计学意义(均P0.05);不同改良Osserman分型亚组中仅全身型重症肌无力(Ⅱa和Ⅱb型)血清抗ACh R抗体阳性率高于眼肌型重症肌无力(Ⅰ型;P=0.005,0.012),而抗Titin抗体和抗Ry R抗体阳性率差异无统计学意义(均P0.05)。结论血清抗ACh R抗体、抗Titin抗体和抗Ry R抗体均有助于诊断重症肌无力,对于3种生物学标志物同时呈阳性的患者,应高度怀疑重症肌无力,其中,眼肌型重症肌无力患者血清抗ACh R抗体水平较高,有可能进展为全身型重症肌无力,应积极治疗。     

EMG evidence of myopathy and the occurrence of titin autoantibodies in patients with myasthenia gravis

We studied 65 patients with myasthenia gravis (MG). Clinical, neurophysiological, immunological, and histological findings suggested the coexistence of a presumed autoimmune myopathy. The clinical features were persistent pyridostigmine-resistant weakness and atrophy of striated muscles. The myopathy was found more often in patients with late-onset MG than in those with early-onset (37% vs 13%). Patients with myopathy were also prone to have other immune disorders (47% vs 13%). Elevated titres of antibodies against titin were detected more often in patients with electromyography (EMG) evidence of myopathy than in the sera of those without, and only in late-onset MG cases. Copyright Lippincott Williams & Wilkins     

Studying the relationship between clinical features and mental health among late-onset myasthenia gravis patients

BACKGROUNDMental disorders are common comorbidities among individuals with neurological diseases, and the prevalence of depressive and anxiety-related symptoms in newly referred patients at neurology outpatient clinics is high. There have been few studies on the mental health of patients with late-onset myasthenia gravis (MG).AIMTo examine the relationship between clinical features and the mental health symptoms within late-onset MG patients.METHODSA total of 105 patients diagnosed with MG were recruited consecutively from a neuromuscular outpatient clinic between December 2020 and February 2021. Patients were classified into two groups: early-onset MG (age at onset < 50 years, n = 63) and late-onset MG (age at onset ≥ 50 years, n = 42). Social demographic data and information about marital status, education level, clinical symptoms, serum antibody levels, and therapies used were collected for all participants. Participants were also evaluated using the Myasthenia Gravis Composite scale, the Myasthenia Gravis Activities of Daily Living scale, the Myasthenia Gravis Quality of Life 15 (MG-QOL-15) questionnaire, the 17-item version of the Hamilton Depression Rating Scale (HAM-D) and the Hamilton Anxiety Rating Scale (HAM-A). The relationship between clinical features and mental health in late-onset MG patients was examined using multivariate logistic regression analyses. RESULTSLate-onset MG patients were more prone to dyspnea, had higher levels of serum anti-acetylcholine receptor antibodies, and higher total scores on the MG-QOL-15, HAM-D, and HAM-A questionnaires, than early-onset MG patients had (P < 0.05). Among those with late-onset MG, female patients had higher total HAM-D and HAM-A scores than male patients had (P < 0.05). High scores on the QOL-15 questionnaire were associated with higher incidences of anxiety and depression, and the association was found to be independent after adjusting for confounding risk factors. In the late-onset subgroup, the areas under the receiver operating characteristic curves for the MG-QOL-15 score-based diagnostic accuracy for anxiety and depression state were 0.816 (P = 0.001) and 0.983 (P < 0.001), respectively.CONCLUSIONHigher MG-QOL-15 scores were a risk factor for anxiety and depression in late-onset MG, and women with late-onset MG were more likely to have anxiety and depression than men were.     

Nonthymoma early-onset- and late-onset-generalized myasthenia gravis--a retrospective hospital-based study

OBJECTIVE: Acquired myasthenia gravis (MG) is predominantly due to nicotinic acetylcholine receptor (AChR) autoantibodies (Ab). Differences between nonthymoma early-onset and late-onset MG were reported. We studied the clinical and serological characteristics of nonthymoma AChR Ab-positive-generalized MG patients. PATIENTS AND METHODS: Chinese AChR Ab-positive-generalized MG patients who had generalized disease for 3 years or longer were studied. RESULTS: Among 41 such patients, 25 (61%) were female. The mean onset age was 43.5 years (range 9-78 years) and the mean follow-up duration was 7.8 years (range 3-20 years). Sixteen (39%) patients had late-onset disease (onset age >or=50 years). Compared to early-onset patients (onset age <50 years), late-onset patients were characterized by male predominance (p=0.002), absence of thymic lymphofollicular hyperplasia (p=0.036), and a higher striated muscle Ab seropositivity rate (94% versus 4%, p<0.001). Although there was no statistically significant difference in clinical severity and outcome or response to treatment between late-onset and early-onset patients, 50% and 75% of late-onset patients had moderate or severe disease at onset and worst status, respectively, compared to 28% and 52% for early-onset patients at onset and worst status, respectively. Also 63% of late-onset patients had disease progressed within first 3 years compared to only 40% of early-onset patients did. CONCLUSION: Nonthymoma late-onset-generalized MG patients were common among Hong Kong Chinese, with a statistically non-significant trend that it was clinically more severe than early-onset MG but with similar clinical outcome or response to treatment; >90% of these patients were seropositive for striated muscle Ab.     

Increasing incidence of late-onset anti-AChR antibody-seropositive myasthenia gravis

The incidence of myasthenia gravis (MG) from 1970 through 1999 was studied in an area with 2.3 million inhabitants. The mean annual incidence rate of early-onset MG was constant at 3.5 x 10(-6). In late-onset MG, the rate increased from 4.7 to 20.8 x 10(-6). The two onset types of MG may thus be distinct disorders. The author hypothesized that late-onset nonthymoma anti-acetylcholine receptor antibody-seropositive MG may be provoked by environmental factors.     

Clinical and immunological differences between early and late-onset myasthenia gravis in Japan

Immunological characteristics of myasthenia gravis (MG) with late-onset have not been fully elucidated. We examined several autoantibodies and HLA-DRB1 genotyping in 260 Japanese MG patients. Sixty-two MG patients had thymoma. The others were divided into early-onset and late-onset groups separated by an age of 50 years. The ocular form was more frequent in late-onset compared to early-onset group. Seropositivity of anti-muscle-specific tyrosine kinase antibody was 2-3% in acetylcholine receptor-seronegative patients. HLA-DRB1 genotyping failed to detect statistical differences in specific alleles between each group and healthy controls. The immunological profiles in late-onset MG were different from early-onset in Japan.     

Characteristics of late-onset myasthenia gravis

An increasing incidence of myasthenia gravis (MG) has been reported in the elderly, but the full clinical ramifications of late-onset myasthenia gravis (LOMG) remain unclear. We describe the clinical features of our cohort of patients with MG with an emphasis on an onset after the age of 50. This was a retrospective analysis of medical records of a cohort of patients followed in two tertiary neuromuscular clinics and comparison of early onset MG (EOMG) versus LOMG. There were 174 patients with a mean age of onset of 55.2?±?19.1?years, and 44 % were women. Late onset of myasthenia gravis after age 50 was reported in 114 patients (66 %). Anti-AChR antibody titers were elevated in 78 % of patients (65 % with EOMG vs. 85 % with LOMG; p?=?0.003), and frequency of elevated titers of anti-MuSK antibodies was similar in both groups (present in 38 % of all tested seronegative patients). Myasthenic crisis was equally common in generalized EOMG and LOMG (13 %). Ocular MG was more common in LOMG compared to EOMG (40 vs. 18 %, p?=?0.021). Diabetes was more prevalent with LOMG (27 vs. 5 %; p?=?0.0002). Overlapping clinical features of EOMG and LOMG are consistent with a continuous clinical spectrum of a single condition, with more frequent occurrence of seropositive and ocular MG with a late onset. A higher burden of comorbidities, such as diabetes mellitus, may warrant a modified approach to treatment of myasthenia in LOMG. However, overall disease severity may not be higher with aging. These observations have implications for design of MG clinical trials and outcomes studies.