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1.
重症肌无力(MG)临床测评量表作为MG病情的量化工具,应用贯穿于MG的治疗和随访全程。近年来,随着循证医学模式的发展及大数据在临床医学领域内的应用,MG测评量表在临床研究和工作中进行定量肌无力程度、评估生活质量、评价药物疗效、监测病情变化及指导随访治疗等方面发挥了重大作用。细数国内外开发出的各种MG测评量表,评价角度多样,内容全面详实,具有很高的实用价值。但由于数量众多,优势不一,给研究人员和临床医生的应用选择造成了困难。为此,文中对近年来国内外临床研究中常用的MG测评量表进行分类介绍,并对量表信度和效度进行总结,旨在为广大临床研究人员和临床医生选择合适的量表提供理论和实践依据。  相似文献   

2.
目的研究重症肌无力(MG)危象发生的危险因素及防治措施。方法对我院2010-03—2015-10收治的100例重症肌无力患者临床资料进行整理、归纳分析,包括患者年龄、激素治疗、胸腺切除与否等,行单因素及多因素Logistic回归分析,总结重症肌无力危象危险因素并提出相应防治措施。结果 MG危象组平均年龄(33.4±10.5)岁,显著低于MG非危象组的(38.8±11.0)岁,差异有统计学意义(P0.05)。MG危象组激素治疗、合并感染、胸腺切除史比例分别为85.7%、57.1%、54.8%,显著高于MG非危象组的56.9%、12.1%、27.6%,差异有统计学意义(P0.05)。多因素Logistic回归分析年龄、激素治疗、合并感染及胸腺切除史是重症肌无力危象发生的独立危险因素。结论激素治疗、合并感染、胸腺切除术史是重症肌无力危象发生危险因素,需规范激素治疗,积极预防感染及严格筛选胸腺切除手术指征。  相似文献   

3.
目的提高临床医师对儿童期以咽喉部肌无力起病的重症肌无力(MG)临床表现的认识水平,做到及时诊断和个体化治疗。方法分析6例以咽喉部肌无力为主要表现的儿童期MG的诊断、鉴别诊断及治疗过程。结果在诊断儿童期MG明确后,给予规范治疗,病情迅速好转。结论以吞咽困难、构音障碍等咽喉肌病变表现为MG唯一或主要起病症状时,应尽早监测甲状腺功能情况,积极干预管理。  相似文献   

4.
重症肌无力(MG)的治疗在文献中已有广泛报导,但对没有胸腺瘤的MG 采用类固醇治疗和胸腺切除的效果尚无对照研究,特别是对老年人的MG尚有许多问题有待解决。本文报道2例晚发型MG 采用胸腺切除获得成功。例1:男61岁,1981年有复视,颌无力,讲话及吞咽困难,肛门括约肌无力。腾喜龙试验阳性(手  相似文献   

5.
<正>重症肌无力(myasthenia gravis,MG)是一种累及神经肌肉接头突触后膜上乙酰胆碱受体的自身免疫性疾病,主要表现为骨骼肌易疲劳,晨轻暮重,虽然该病发病率相对较低,但是具有病程长、波动性大、有发生危象潜在可能性等特点,并对于个体化治疗要求高。目前,国内外针对MG的病因、发病机制、诊治等方面研究多为小样本量研究,统计分析数据证据力度有限。通过临床注册登记所收集的数据符合医学大  相似文献   

6.
赵经纬  王强 《中国卒中杂志》2016,11(12):1086-1088
作为确立并不久的重症医学的一部分,神经重症医学,更是一个新兴的亚专科。其研究对象主要包括重症脑血管病、颅脑外伤、脑肿瘤及其他神经系统相关重症患者。其理论体系尚不完善,很多大型随机对照研究结论存在相悖的现象。这使得初入该领域的年轻医生有时在临床实践中无所适从。为解决这一问题,笔者希望通过对近年流行的循证医学理念进行反思,强化科学精神,并通过引入证据链概念,帮助神经重症专业的年轻医生解读纷繁复杂的循证医学证据,并最终建立正确的临床思维方法。  相似文献   

7.
重症肌无力(myasthenia gravis,MG)是由抗体介导、细胞免疫依赖、补体参与的自身免疫性疾病,胸腺在诱导和维持MG发病、发展过程中起重要作用。随着循证医学研究的深入和微创外科技术的进步,胸腺切除已被公认为治疗MG的有效手段,并快速进入微创时代。MG外科领域仍存留部分未解难题,包括手术适应证和时机选择、不同手术入路和清扫范围之间的疗效差别等。本文旨在回顾MG外科发展历史,对MG外科治疗的理论基础、手术方法及进展进行综述。  相似文献   

8.
重症肌无力(MG)是一种由自身抗体介导的神经-肌肉接头(NMJ)信号传递障碍的获得性自身免疫性疾病。自身抗体检测是国内外MG诊治指南推荐的关键辅助诊断指标。MG自身抗体的检测方法包括放射免疫沉淀法、酶联免疫吸附测定法和细胞免疫荧光法等, 研究表明这些检测方法的特异度和敏感度存在客观差异, 需要结合临床诊断需求来选择不同的检测方法学。为此, 中华医学会神经病学分会神经免疫学组基于国内外MG自身抗体诊断指南、检测技术研究进展和充分征求学组专家意见的基础上形成《重症肌无力自身抗体实验室诊断专家共识2022》。本专家共识对MG自身抗体的检测方法学选择给出指导性建议, 进一步促进我国MG自身抗体诊断的规范化。  相似文献   

9.
由美国重症肌无力基金会(MGFA)的医学科学建议委员会(MSAB)组成工作组,于2010年在美国和2011年在意大利召开专家共识会议讨论重症肌无力(MG)临床试验的问题,与会代表包括来自美国和欧洲的神经科医生、统计学家、企业界代表、MG患者以及MGFA和欧洲重症肌无力协会的平民代表. 会议总结以往的MG临床试验存在的问题包括:(1)大多数试验入组患者的病情发展较预期明显缓慢;(2)单独使用泼尼松改善肌无力症状的疗效可能比预期明显,所以较难发现联用免疫抑制剂而产生的疗效;(3)为减少激素剂量,大多数的临床试验时间过短,难以发现疗效;(4)入组标准太窄,在结果推广时会产生困难;(5)预后指标不够敏感或定义的太窄以至于无法发现重要的临床效应.  相似文献   

10.
目的研究重症肌无力(MG)危象的有效治疗方法,提高MG危象抢救的成功率和缩短机械通气的时间。方法应用静脉注射丙种球蛋白(IVIG)、甲基强的松龙、环孢霉素A及机械呼吸治疗3例重症肌无力危象患者,根据临床症状判断疗效。结果3例MG危象患者症状缓解,随诊6个月~2年,3例可以正常生活。结论IVIG、甲基强的松龙、环孢霉素A联合治疗加机械呼吸是治疗MG危象的有效措施。  相似文献   

11.
The relationship between myasthenia gravis (MG) and epilepsy has been rarely reported. As consequence, there are no specific guidelines for the management of these conditions when they mutually occur. We reported on three patients in whom epilepsy and MG are coexisting, but in different clinical settings. Two patients were treated with antiepileptic drugs which improved their symptoms. One patient has controlled the seizures after a successful anterior temporal lobectomy with no appreciable consequences to her MG. We discuss the difficulties in the management of epilepsy in patients with MG. In addition, we report on the first epileptic surgery in a MG patient, indicating that this surgical procedure as a safe option for the treatment of intractable epilepsy in patients with MG.  相似文献   

12.
The clinical course of myasthenia gravis (MG) is variable, and spontaneous remission is still uncommon. Knowledge of the prognostic factors may help understand the course of MG and thus optimize its management. A systematic review search was conducted in MEDLINE and EMBASE for English language studies from 1985 through 2009. We identified additional studies by reviewing bibliographies of retrieved articles and hand search main journal of neurology. Studies evaluating variables associated with or predictive of remission in adult patients with MG were included. Because of methodological heterogeneity, we refrained from statistical pooling, instead, a best evidence synthesis was used for summarizing the results. From 1810 potentially relevant studies, 13 cohort studies met the inclusion criteria. The included studies were heterogeneous considerably in sample size, disease duration, follow‐up years, definition of remission, and analysis. Study quality was limited by retrospective design in most studies and lack of multivariate analysis. Time of diagnosis from onset (<1 year) showed strong evidence of predicting a better remission. In studies using completely stable remission outcomes, there was strong evidence that age at onset (<40 years) was of prognostic importance. Furthermore, gender showed no association with remission. Time of diagnosis from onset and age at onset were found to be predictors of remission. Gender does not seem to predict the course of MG. Our findings should be interpreted with caution because of the clinical and methodological heterogeneity of included studies.  相似文献   

13.
Growing evidence provides new insights about myasthenia gravis (MG) with antibodies against muscle‐specific tyrosine kinase (MuSK‐MG), including its pathogenesis, clinical and electrophysiological manifestations, and treatment. Data now support the presence of both presynaptic and postsynaptic dysfunction in MuSK‐MG. This is 1 of many key differences between MuSK‐MG and acetylcholine receptor antibody‐MG (AChR‐MG), especially as it pertains to potential therapeutic implications. In comparison to AChR‐MG, MuSK‐MG is generally more refractory to treatment. However, because MuSK‐MG is better understood and more readily recognized today, there are more reports of a relatively benign course. The most effective immunotherapies for MuSK‐MG are corticosteroids, plasmapheresis, and rituximab. With appropriate therapy, most patients with MuSK‐MG achieve minimal manifestation status or better on the postintervention status outlined by the Myasthenia Gravis Foundation of America. A minority of patients remain refractory to treatment, and optimal management for this group remains a considerable challenge. Muscle Nerve 58 : 344–358, 2018  相似文献   

14.
The prevalence of myasthenia gravis (MG) in the counties of Hordaland and Sogn & Fjordane on January 1, 1984 was 9.6 per 100,000 inhabitants. Other autoimmune diseases were found in 11 out of 48 MG patients. The occurrence of autoimmune thyroiditis (5 patients, 10.4%) and systemic lupus erythematosus (4 patients, 8.3%) in the MG patients was clearly higher than that reported in the general population. Rheumatoid arthritis was found in 2 patients (4.2%). The autoimmune diseases were mainly recorded among the nonthymectomized MG patients. In addition to those with definite diseases of autoimmune nature, 3 other MG patients had thyroid antibodies and 1 had antinuclear factor without clinical evidence of autoimmune disease. Seven MG patients (14.6%) had unspecific arthralgia during active periods of MG. Two MG patients had ankylosing spondylitis.  相似文献   

15.
Subcutaneous immunoglobulin (SCIg) is an emerging therapeutic alternative in the management of myasthenia gravis (MG) due to its potential efficacy, safety, cost effectiveness and ease of administration. At present, there are no systematic reviews that summarized the effects of SCIg in patients with MG. The objective of this study is to determine the efficacy and safety of SCIg in the treatment of adult patients with myasthenia gravis. Relevant records were identified from August 2018 to January 2019 systematic search. Five relevant articles with a total of 34 patients with MG were included in this review. Data on functional disability score and adverse events were obtained. Based on the included uncontrolled studies, the functional disability scores of adult MG patients after SCIg administration showed consistent improvement. Headache and local site injection reactions were the most common adverse events reported. The evidence from limited uncontrolled studies gathered in this review showed that SCIg may improve functional disability in patients with MG. Local and mild adverse events were reported with its administration, but no systemic and serious adverse events were noted.  相似文献   

16.
Prediction of the risk of dysphagia and aspiration is important in the management of myasthenia gravis (MG). We assessed the ability of four bedside clinical tools to predict aspiration in 20 MG patients. Patients completed a self-directed questionnaire, underwent clinical neurological assessment and a bedside speech pathology assessment, and were assessed with the quantitative myasthenia gravis (QMG) score. The ability of these tools to predict aspiration was compared with the results of a modified barium swallow. Seven patients aspirated, 4 silently. The total self-directed questionnaire score, two specific questions on the self-directed questionnaire, the prediction based on clinical neurological assessment, and the QMG bulbar subset score all correlated with aspiration. The speech pathology prediction was highly sensitive but less specific. This pilot study shows that simple clinical tools can predict which MG patients are at risk of aspiration.  相似文献   

17.
Thymectomy is a frequently used treatment for myasthenia gravis (MG) and is virtually always indicated in MG patients who have a thymoma. However, the evidence for thymectomy in non-thymomatous MG remains less certain—no randomised controlled trials have been published to date, although one is currently underway. We reviewed the management and clinical outcome of patients with MG who underwent thymectomy over a 12 year period. Eighty-nine patients who underwent transsternal thymectomy were identified. A thymoma was identified on histology in 24 %, whereas 48, 9 and 19 % had hyperplastic, atrophic and normal thymic histology, respectively. One patient developed post operative myasthenic crisis but generally the procedure was well tolerated. Outcome was favourable for the majority of patients, with 34 % achieving complete stable remission (CSR) and an additional 33 % achieving pharmacological remission. Moreover, steroid requirements fell progressively during follow-up. Patients with a hyperplastic gland had a significantly greater chance of achieving CSR compared to other histological subtypes and the incidence of CSR increased with a longer duration of follow-up. Thymectomy for MG is generally safe and well tolerated and is associated with a sustained improvement of symptoms in the majority of patients.  相似文献   

18.
Patients with myasthenia gravis (MG) are divided into 3 groups: anti-acetylcholine receptor antibody-positive MG (AChR MG), anti-muscle specific kinase antibody-positive MG (MuSK MG), and AChR-and MuSK-negative MG (double seronegative MG; double SNMG). A recent study on the detection of low-affinity antibodies binding to AChR showed the presence of AChR antibodies in about 70% of double SNMG patients. There is accumulating evidence that double SNMG patients are similar to AChR-MG patients with respect to clinical features and thymic pathology. Since most MG patients are thought to belong to the AChR-MG or MuSK-MG group of patients, this article reviews the pathophysiology, clinical features, and treatments in these 2 groups of MG patients. The pathophysiology of AChR-MG is closely related to the abnormal thymic pathology, such as thymic hyperplasia or presence of thymoma, and thy(mo)mectomy is recommended in patients with generalized AChR-MG. On the contrary, little thymic abnormality in patients with MuSK-MG discourages thymectomy; however, MuSK-MG patients do respond to thymectomy and therefore studies to define the indications of thymectomy in MuSK-MG patients are required. The responses to cholinesterase inhibitors are poor in patients with MuSK-MG, and these patients tend to show hyperactivity to the Tensilon test, such as fasciculation of facial muscles and stuffy sensation in the throat. The adverse responses to a small dose of intravenous edrophonium chloride injection may support the clinical diagnosis of MuSK-MG. Further, only small doses of acetylcholinesterase inhibitors are administered to patients with MuSK-MG in order to avoid cholinergic hyperactivity. In general, both types of MG patients respond well to treatments with immunosuppressants, including steroids, but some patients with MuSK-MG show persistent bulbar symptoms.  相似文献   

19.
Myasthenia gravis (MG) is an antibody-mediated, neuromuscular transmission disorder, which ranges in clinical manifestations of ocular myasthenia that may be visually disabling to myasthenic crisis with patients suffering life-threatening respiratory insufficiency. MG also has pathophysiologic subgroups based on presence or absence of acetylcholine receptor or muscle-specific kinase antibodies and presence of thymoma. Cholinesterase inhibitors partially improve weakness, but the vast majority of patients require therapies that moderate the autoimmune attack. Mortality of MG has been reduced over the last century, but adverse effects of treatment compromise patient care and rigorous evidence to guide the clinician are lacking.  相似文献   

20.
OBJECTIVE: To report the clinical, electrophysiological, and immunological findings in one patient with MG and LEMS overlap syndrome (myasthenia gravis and Lambert-Eaton myasthenic syndrome). METHODS: Evaluation of clinical, electrophysiological, and immunological findings in one patient with this disease. RESULTS: A female patient with pernicious anemia had clinical findings of ptosis, diplopia, proximal leg weakness, areflexia and a positive edrophonium test as well as the classic patterns initially of MG and later of LEMS on successive repetitive nerve stimulation (RNS) tests. Immunologically she demonstrated positive acetylcholine receptor antibody (ACh-ab) and positive N-type voltage-gated calcium-channel antibody (VGCC-ab) titers. CONCLUSIONS: Among five reported cases combining the features of MG and LEMS and having positive AChR- and VGCC-ab titers, our patient provides the most convincing clinical, electrophysiological, and immunological evidence for the existence of MG and LEMS overlap syndrome. Significance: MG and LEMS overlap syndrome is a distinct entity.  相似文献   

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