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Possible implications of dysregulated nicotinic acetylcholine receptor diffusion and nanocluster formation in myasthenia gravis

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Neural Regeneration Research 2021年第2期16卷 242-246页

作者： Francisco J.Barrantes Biomedical Research Institute(BIOMED)UCA-CONICET Buenos AiresArgentina

myasthenia gravis is a rare and invalidating disease affecting the neuromuscular junction of voluntary *** classical form of this autoimmune disease is characterized by the presence of antibodies against the most abundant protein in the neuromuscular junction,the nicotinic acetylcholine *** variants of the disease involve autoimmune attack of non-receptor scaffolding proteins or enzymes essential for building or maintaining the integrity of this peripheral *** review summarizes the participation of the above proteins in building the neuromuscular junction and the destruction of this cholinergic synapse by autoimmune aggression in myasthenia *** review also covers the application of a powerful biophysical technique,superresolution optical microscopy,to image the nicotinic receptor in live cells and follow its motional *** hypothesis is entertained that anomalous nanocluster formation by antibody crosslinking may lead to accelerated endocytic internalization and elevated turnover of the receptor,as observed in myasthenia gravis.

关键词： agrin autoimmune diseases muscle end-plate muscle specific kinase MuSK myasthenia gravis nanoscopy neuromuscular junction nicotinic acetylcholine receptor rapsyn superresolution microscopy

Adult celiac disease with acetylcholine receptor antibody positive myasthenia gravis

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World Journal of Gastroenterology 2009年第38期15卷 4741-4744页

作者： Hugh J Freeman Helen R Gillett Peter M Gillett Joel Oger Department of Medicine (Gastroenterology and Neurology) University of British Columbia

Celiac disease has been associated with some autoimmune disorders. A 40-year-old competitive strongman with celiac disease responded to a gluten-free diet, but developed profound and generalized motor weakness with acetylcholine receptor antibody positive myasthenia gravis, a disorder reported to occur in about 1 in 5000. This possible relationship between myasthenia gravis and celiac disease was further explored in serological studies. Frozen stored serum samples from 23 acetylcholine receptor antibody positive myasthenia gravis patients with no intestinal symptoms were used to screen for celiac disease. Both endomysial and tissue transglutaminase antibodies were examined. One of 23 (or, about 4.3%) was positive for both IgA-endomysial and IgA tissue transglutaminase antibodies. Endoscopic studies subsequently showed duodenal mucosal scalloping and biopsies confirmed the histopathological changes of celiac disease. Celiac disease and myasthenia gravis may occur together more often than is currently appreciated. The presence of motor weakness in celiac disease may be a clue to occult myasthenia gravis, even in the absence of intestinal symptoms.

关键词： Acetylcholine receptor antibodies Celiacdisease myasthenia gravis Transglutaminase antibodies

Computer Aided Study of Mechanism of Immunoadsorbent for myasthenia gravis

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Chinese Chemical Letters 2003年第7期14卷 693-696页

作者： Wei Rong YAN Yao Ting YU Nan DONG The Key Laboratory of Bioactive Materials Nankai UniversityTianjin 300071

myasthenia gravis (MG) is an organ specific autoimmune disease mediated by autoantibodies (AChR Ab) against the acetylcholine receptor (AChR). Literature reported that the AChR Ab can be removed by absorbent linked with tryptophan. It was studied in detail in our lab. With the aid of computer, we docked some ligands into AChR Ab, and the results from scores of docking under different generations showed that there was no specific binding between tryptophan and scFv fragment just as the binding between antigen-antibody. The interaction between Trp and immunoglobulin was a broad-spectrum binding.

关键词： myasthenia gravis docking SYBYL6.6.

Jitter analysis with concentric needle electrodes in the extensor digitorum communis for the diagnosis of myasthenia gravis：a pilot study

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Chinese Medical Journal 2014年第18期127卷 3209-3212页

作者： Wang Jianwen Ji Fang Luo Benyan Department of Neurology First Affiliated Hospital Zhejiang University School of Medicine Hangzhou Zhejiang 310003 China

Background Single-fiber electromyography is the most sensitive neurophysiological test for the diagnosis of myasthenia gravis （MG）,but its use is limited by the potential risk of transmission of *** analysis with disposable concentric needle electrodes （CNEs） is therefore being *** pilot study aimed to evaluate jitter analysis with CNEs for the diagnosis of *** Forty-two healthy Chinese volunteers and 44 MG patients were prospectively *** patients were classified according to the Osserman classification,and acetylcholine receptor antibody titer was *** analysis with CNEs in the extensor digitorum communis and repetitive nerve stimulation （RNS） testing were *** was expressed as the mean consecutive difference （MCD）,and 20 action potential pairs were analyzed in each *** mean MCD in each subject and the mean individual MCD of all action potential pairs were compared between *** The mean MCD and mean individual MCD were higher in MG patients （（42.3±20.0） μs and （42.2±26.0） μs） than in healthy volunteers （（23.0±3.1） μs and （22.8±7.5） μs）.The area under the receiver operating characteristic curve for the mean MCD of MG patients and healthy volunteers combined was *** mean MCD and mean individual MCDwere higher in generalized MG patients （（64.1±18.5） μs and （63.6±30.0） μs） than in ocular MG patients （（33.1±12.0） μs and （33.2±17.6） μs）,and were higher in MG patients with abnormal RNS results （（57.2±18.3） μs and （57.3±29.2） μs） than in MG patients with normal RNS results （（32.9±14.8） μs and （32.7±18.3） μs）.Abnormal RNS results were observed in 38.60% （17/44） of MG patients and abnormal jitter were observed in 72.70% （32/44） of MG *** Jitter analysis with CNEs is feasible for the diagnosis of MG.

关键词： myasthenia gravis single fiber electromyography,'jitter concentric needle electrode

Long-term prognostic analysis of thymectomized patients with myasthenia gravis

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Chinese Medical Journal 2002年第2期115卷 235-237,153页

作者：刘伟佟倜纪振东张振和白求恩医科大学第一临床学院胸外科长春130021

OBJECTIVE: To study the factors affecting the long-term prognosis of patients with myasthenia gravis (MG) after thymectomy. METHODS: 170 MG patients who had undergone thymectomies were studied retrospectively. Among them, 124 patients received long-term follow-up for more than 40 months postoperatively. The COX regression analysis model was used to analyze the factors that may influence the long-term prognosis. These factors included thymus pathology, patient gender, age, duration of disease at the time of surgery, preoperative Osserman classification and medication. RESULTS: The research showed that thymus pathology was the single independent factor that affected the postoperative long-term prognosis. The long-term survival rates differed significantly with thymus pathological types: hyperplasia > benign thymoma > atrophy > malignant thymoma (P

关键词： Adolescent Adult Aged Atrophy Child Child, Preschool Female Humans Hyperplasia Male Middle Aged myasthenia gravis Postoperative Complications Prognosis Research Support, Non-U.S. Gov't Survival Analysis Survival Rate Thymectomy Thymoma Thymus Gland Time Factors

Detection of multiple antibodies in myasthenia gravis and its clinical significance

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Chinese Medical Journal 2010年第18期123卷 2555-2558页

作者： WANG Wei-wei HAO Hong-jun GAO Feng Department of Geriatrics Beijing Jishuitan Hospital Beijing 100035 China Department of Neurology Peking University First Hospital Beijing 100034 China

Background Antibodies against acetylcholine receptor, acetylcholinesterase, ryanodine receptor and titin have been found in patients with myasthenia gravis. However, the relations between these antibodies and character of myasthenia gravis are unknown. This study aimed to detect multiple antibodies in myasthenia gravis and to investigate its clinical significance. Methods These antibodies were detected by enzyme-linked immunoabsorbent assay in 89 cases of myasthenia gravis, 66 cases of other neurological diseases and 66 healthy controls. The incidences of antibodies were compared using the chi-square test. Results Acetylcholine receptor, acetylcholinesterase, titin and ryanodine receptor antibodies were detected in 53.9%, 20.2%, 64.0% and 55.0% of myasthenia gravis patients respectively, higher than in patients of other neurological diseases and controls groups. The combination of the four antibodies assays provided 94.4% sensitivity and 84.0% specificity for the diagnosis of myasthenia gravis. Acetylcholinesterase antibody occurred more frequently in acetylcholine receptor antibody negative patients with adverse reactions to neostigmine test. Titin antibody provided 82.1% sensitivity and 52.5% specificity for myasthenia gravis with thymoma. Incidences of titin and of ryanodine receptor antibody were higher in late onset myasthenia gravis than in early onset myasthenia gravis. The proportion of titin antibody positive patients increased with the severity of myasthenia gravis as graded by a modified Osserman scale. Conclusions Testing for acetylcholine receptor, acetylcholinesterase, titin and ryanodine receptor antibodies can offer a better diagnostic method for myasthenia gravis than each antibody test alone. Titin antibody combined with computed tomography was better for the diagnosis of thymoma. Titin antibody occurred most frequently in severe myasthenia gravis.

关键词： myasthenia gravis thymoma antibody

FcRn inhibitors:a novel option for the treatment of myasthenia gravis

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Neural Regeneration Research 2023年第8期18卷 1637-1644页

作者： Li-Na Zhu Hai-Man Hou Sai Wang Shuang Zhang Ge-Ge Wang Zi-Yan Guo Jun Wu Department of Neurology The First Affiliated Hospital of Zhengzhou UniversityZhengzhouHenan ProvinceChina

myasthenia gravis is an acquired,humoral immunity-mediated autoimmune disease characterized by the production of autoantibodies that impair synaptic transmission at the neuromuscular *** intervention-mediated clearance of immunoglobulin G(IgG)was shown to be effective in controlling the progression of the *** neonatal Fc receptor(FcRn)plays a key role in prolonging the serum half-life of *** FcRn to prevent its binding to IgG can accelerate the catabolism of the latter,resulting in decreased levels of IgG,including pathogenic autoantibodies,thereby achieving a therapeutic *** this review,we detail the substantial research progress,both basic and clinical,relating to the use of FcRn inhibitors in the treatment of myasthenia gravis.

关键词： batoclimab clinical trial efgartigimod FcRn FcRn inhibitors immunoglobulin G(IgG) myasthenia gravis nipocalimab rozanolixizumab

Effectiveness of Thymectomy in Non-thymomatous myasthenia gravis: a Systematic Review

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Journal of Huazhong University of Science and Technology(Medical Sciences) 2014年第6期34卷 942-949页

作者：罗彦潘邓记陈菲菲朱明辉王晶张旻 Department of Neurology Tongji Hospital Tongji Medical College Huazhong University of Science and Technology

There is continuous debate regarding the effectiveness of thymectomy in the treatment of non-thymomatous myasthenia gravis （MG）. This systematic review was undertaken to determine whether thymectomy was effective in non-thymomatous MG. We retrieved articles published between January 1980 and September 2013. Sixteen cohort studies were included. Given the considerable het- erogeneity, we used a descriptive method instead of statistical synthesis. The median relative rates （RRs） and their interquartile ranges were used to estimate the magnitude of benefit. Compared to conserva- tively treated MG patients, thymectomized patients had higher survival, clinical remission, pharma- cologic remission and improvement rates, and RRs were 1.07 （1.01-1.17）, 1.83 （0.82-2.99）, 1.55 （1.22-1.95） and 1 （1.00-1.09）, respectively. Subgroup analyses showed that patients with moderate to severe generalized MG benefited more from thymeetomy, with RRs of survival and pharmacologic re- mission increasing to 1.35 （1.24-1.49） and 2.68 （1.73-4.17）, respectively. These results suggested that thymectomy might be an effective procedure in non-thymomatous MG patients. The patients with mod- erate to severe generalized MG might benefit more. Taking into account the poor methodological qual- ity of present studies, more well-designed prospective randomized controlled trials （RCTs） are still re- quired to reach uneouivocal conclusion.

关键词： myasthenia gravis thymectomy systematic review

Evaluation of A Scale of Patient-Reported Outcomes for the Assessment of myasthenia gravis Patients in China

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Chinese Journal of Integrative Medicine 2012年第10期18卷 737-745页

作者：刘凤斌陈新林郭丽刘小斌 Department of Internal Medicine The First Affiliated HospitalGuangzhou University of Chinese Medicine College of Basic Medical Science Guangzhou University of Chinese Medicine The Second Hospital Liwan District of Guangzhou The DENG Tietao Institute Guangzhou University of Chinese Medicine

Objective： To evaluate a scale of patient-reported outcomes for the assessment of myasthenia gravis patients （MG-PRO） in China. Methods： A total of 100 MG patients were interviewed for the field testing. Another 56 MG patients were selected and assessed with the MG-PRO scale before treatment and at 1, 2 and 4 weeks after treatment. The classical test theory and item response theory （IRT） were used to assess the psychometric characteristics of the MG-PRO scale, Results： The MG-PRO scale included 4 dimensions： physical, psychological, social environment, and treatment. Confirmatory factor analysis showed that each dimension was consistent with the theoretical construct. The scores of the physical and psychological dimensions increased significantly at 1 week after treatment （P〈0.05）. All the dimension scores and the MG-PRO score increased significantly at 2 and 4 weeks after treatment （P〈0.05）. IRT showed that person separation indices were greater than 0.8, most of the item fit residual statistics were within ＋ 2.5, and no item had uniform or non-uniform differential item functioning （DIF） between gender and age （〈40, 〉140）. Conclusions： The MG-PRO scale is valid for measuring the quality of life （QOL） of MG patients, with good reliability, validity, responsiveness, and good psychometric characteristics from IRT. It can be applied to evaluate the QOL of MG patients and to assess treatment effects in clinical trials.

关键词： myasthenia gravis quality of life patient-reported outcome classical test theory item response theory