Functional exercise capacity evaluated by timed walk tests in myasthenia gravis

Introduction: We sought to evaluate the test–retest reliability and construct validity of the 6- and 2-minute walk tests (6mWT and 2mWT, respectively) in patients with myasthenia gravis (MG). Methods: Thirty-one patients with generalized MG were enrolled in this study. The 6mWT, 2mWT, MG-specific quality of life questionnaire Turkish version (MG-QoL15T), quantitative myasthenia gravis test (QMG), and pulmonary function tests were administered. Results: The intraclass correlation coefficients of 2mWT and 6mWT were 0.894 and 0.932, respectively. The 6mWT and 2mWT had moderate correlations with forced vital capacity, maximal inspiratory pressure, QMG score, and MG-QoL15T score (ρ for 6mWT: 0.579, 0.539, −0.572, and −0.474; ρ for 2mWT: 0.460, 0.446, −0.532, −0.457). Both tests had similar performances for predicting disease severity (area under the curve = 0.761 for 6mWT and 0.759 for 2mWT). Discussion: The 6mWT and 2mWT have excellent test–retest reliability as well as moderate construct validity for the evaluation of functional exercise capacity patients with MG. Muscle Nerve 59 :208–212, 2019     

补脾益肾方联合温针灸治疗重症肌无力疗效及对患者免疫功能的影响

目的:探讨补脾益肾方联合温针灸治疗对重症肌无力(MG)疗效及对免疫功能的影响。方法:随机分配84例MG病例为西药组和针药组,每组各42例,西药组给予常规西药治疗,即泼尼松片中剂量冲击,小剂量隔日维持治疗,针药组基于以上用药基础给予补脾益肾方联合温针灸治疗,治疗3个月后,统计两组治疗前后的中医证候积分,评估两组中医证候疗效,对比治疗前后的颈部血管流速、T淋巴细胞亚群水平和血清可溶性白细胞介素6受体水平。结果:治疗后,两组中医证候积分显著降低,针药组的变化幅度大于西药组(P<0.05); 针药组的中医证候总有效率低于西药组(P<0.05); 治疗后,两组颈内动脉(ICA)、颈总动脉(CCA)、颈外动脉(ECA)显著提高(P<0.05),两组T淋巴细胞中CD₃⁺、CD₄⁺亚群所占比和CD₈⁺、CD₄⁺比值显著降低(P<0.05),两组血清slL-6R水平均显著降低(P<0.05),以上指标针药组变化幅度大于西药组(P<0.05)。结论:补脾益肾方联合温针灸治疗能缓解MG患者的中医证候症状,提高疗效,促进其颈部血管循环,纠正患者自身机体免疫功能紊乱。     

No todo es lo que parece: miastenia gravis,una rara manifestación paraneoplásica del cáncer de ovario: caso clínico

Paraneoplastic neurological syndromes are an unusual diseases rarely associated with gynaecological tumours. Their presence must alert on the possible existence of an underlying malignant tumour.A rare case is presented of myasthenia gravis as a paraneoplastic manifestation of an advanced primary ovarian cancer.     

重症肌无力患者认知功能特征

目的探讨重症肌无力(myasthenia gravis,MG)患者的认知功能特征。方法纳入83例MG患者和39例健康对照者,应用简易智能状态检查量表(mini-mental status examination,MMSE)、加利福尼亚语言学习测验(California verbal learning test,CVLT)、简易视觉空间记忆测验(brief visuospatial memory test-revised,BVMT-R)、符号数字模式测验(symbol digit modalities test,SDMT)、Benton线方向判断测验(benton judgment of line orientation test,BJLOT)、定步调听觉连续加法测验(paced auditory serial addition test,PASAT)、言语流畅性测验(verbal fluency test,VFT)进行认知功能评估,应用贝克抑郁测验(Beck depression inventory,BDI)评估抑郁状态,对两组资料进行对比分析,并进一步评估疾病分型、合并症情况、病程、疾病严重程度、药物治疗情况等临床特征对MG组认知损害的影响。结果MG组MMSE[28(26,29)分,29(28,30)分]、CVLT、BVMT-R、SDMT[(37.06±12.18)分,(47.54±14.91)分]、PASAT[(32.86±10.23)分,(37.00±8.82)分]评分低于对照组,差异具有统计学意义(P<0.05),两组间BJLOT、VFT、BDI评分差异无统计学意义(均P>0.05)。Spearman相关分析显示重症肌无力患者疾病严重程度定量评分(quantitative MG score,QMG)与SDMT得分存在负相关(r=-0.234,P<0.05);MG日常生活质量量表(MG-activities of daily living profile,MG-ADL)评分与BVMT-R 3试总和得分存在负相关(r=-0.283,P<0.05)。结论重症肌无力存在认知功能损害,主要表现在记忆力、注意力、信息处理速度、视觉记忆,提示重症肌无力可能存在中枢神经系统受累。部分认知领域的损害可能与疾病严重程度相关。临床医生在工作中应加强对MG患者认知障碍的认识,早期评估,密切随访,适当干预。     

Respiratory disease in pregnancy

Physiological changes to the respiratory system in pregnancy make breathlessness a common symptom. However, it is still important to investigate symptoms of breathlessness to exclude pathological causes. This review illustrates some unusual cases of women presenting with breathlessness in pregnancy. Prompt recognition, investigation and management of these women is essential as delays can impact the mother and the neonate. Investigations should be used appropriately and not withheld or delayed due to pregnancy. Effects of respiratory conditions on pregnancy and the neonate are also discussed.     

重症肌无力相关抗体的研究进展

作为一种累及神经肌肉接头的自身免疫性疾病,重症肌无力(myasthenia gravis,MG)的发病机制与自身免疫性抗体密切相关。关于MG相关抗体,研究最多且最为明确的是乙酰胆碱受体抗体(acetylcholine receptor antibody,AChR-Ab),由于存在AChR-Ab阴性的MG患者,近年来学者们开始热衷于研究AChR-Ab以外的其他抗体,如肌肉特异性酪氨酸激酶抗体、低密度脂蛋白4抗体等,并进行相应治疗方案的探索。该文对MG相关抗体的研究进展进行综述,为今后治疗该病提供临床依据和参考。     

重症肌无力患者血清Titin抗体和RyR抗体的临床研究

目的探讨重症肌无力（MG）患者血清Titin抗体和Ryanodine受体（RyR）抗体的临床意义。方法采用ELISA法检测61例MG患者和35例健康体验者（正常对照组）的血清Titin抗体、RyR抗体、AChR抗体。结果MG组Titin抗体和RyR抗体阳性率均显著高于对照组（均P＜0.001）；合并胸腺瘤MG（MGT）组患者Titin抗体阳性率明显高于未合并胸腺瘤MG（NTMG）组（P＜0.01）；MGT组和NTMG组患者RyR抗体阳性率差异无统计学意义（P＞0.05）；晚发型MG患者中Titin抗体和RyR抗体阳性率均明显高于早发型MG患者（均P＜0.05）；全身型或重症型（ⅡA、ⅡB、Ⅲ、Ⅳ型）Titin抗体阳性率显著高于眼肌型（Ⅰ型）（P＜0.01），而两组间RyR抗体阳性率未见统计学差异（P＞0.05）；MG患者Titin抗体和RyR抗体水平与肌无力严重程度相关分析呈正相关（r=0.520、0.487，均P＜0.01）。结论Titin抗体和RyR抗体检测有助于MG的诊断，且Titin抗体有助于鉴别MG患者是否伴随胸腺瘤及进行Osserman分型。Titin抗体与RyR抗体水平还与MG患者病情的严重程度相关，可应用于评估MG患者的病情与预后。