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 ac...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.展开更多
Anti-acetylcholine receptor antibodies （AAR） are considered pathognomonic and pathogenetic for myasthenia gravis （MG）. AAR detection confirms clinical diagnosis of MG. However, AAR is rarely detected in patients w...Anti-acetylcholine receptor antibodies （AAR） are considered pathognomonic and pathogenetic for myasthenia gravis （MG）. AAR detection confirms clinical diagnosis of MG. However, AAR is rarely detected in patients without MG. The underlying pathophysiological mechanisms in a normal subject without MG have not been adequately addressed in previous studies. The present study reports on a case study of a healthy, elderly man with high AAR titers for 14 years. Pathophysiological mechanisms could be due to AAR heterogeneity in specificity, affinity, and multiform, and to muscle variability in response to AAR.展开更多
Anti-bungarotoxin anti-serum,which has the internal image of nicotinicacetylcholine receptor,was used as a tool to measure anti-idiotypic antibodies toantibodies to Iigand of nicotinic acctylcholine receptor in scra f...Anti-bungarotoxin anti-serum,which has the internal image of nicotinicacetylcholine receptor,was used as a tool to measure anti-idiotypic antibodies toantibodies to Iigand of nicotinic acctylcholine receptor in scra from 81 patients withmyasthenia gravis.Enzyme-linked immunosorbcnt assay was adopted.Thc positive ratewas 46.9%(38/81).The specific cross inhibitory test with nicotinic acetylcholinereceptor was positive.Anti-idiotype antibodies to antibodies to ligand of nicotinicacetylcholine receptor in sera of different types of myasthenia gravis patients classified ac-cording to modified Osserman’s standard and myasthenia gravis patients with or withoutthymoma were comparcd in this study and the role of anti-idiotype antibodies toantibodies to Iigand of nicotinic acctylcholinc receptor in the immunity of myasthcniagravis and the possibility of thcrapeutic use of anti-idiotype antibodies arc discussed.展开更多
The antibodies against acetylcholine receptor AchR and levels of SOD and LPO were measured in 11 patients with myasthenia gravis (MG),and the results were compared with normal controls and patients with diseases other...The antibodies against acetylcholine receptor AchR and levels of SOD and LPO were measured in 11 patients with myasthenia gravis (MG),and the results were compared with normal controls and patients with diseases other than MG.The results showed that the antibodies against AchR were higher as compared with other groups before and after operation. The post-operative level of antibodies was obviously lower than the pre-operative value. An slight increase in SOD and significant decrease in mean value of LPO after surgery were noted. The possible mechanism was discussed.展开更多
Single-chain variable domain fragment （scFv） 637 is an antigen-specific scFv of myasthenia gravis. In this study, scFv and human serum albumin genes were conjugated and the fusion pro-tein was expressed in Pichia pa...Single-chain variable domain fragment （scFv） 637 is an antigen-specific scFv of myasthenia gravis. In this study, scFv and human serum albumin genes were conjugated and the fusion pro-tein was expressed in Pichia pastoris. The afifnity of scFv-human serum albumin fusion protein to bind to acetylcholine receptor at the neuromuscular junction of human intercostal muscles was detected by immunolfuorescence staining. The ability of the fusion protein to block myas-thenia gravis patient sera binding to acetylcholine receptors and its stability in healthy serum were measured by competitive ELISA. The results showed that the inhibition rate was 2.0-77.4%, and the stability of fusion protein in static healthy sera was about 3 days. This approach suggests the scFv-human serum albumin is a potential candidate for speciifc immunosuppressive therapy of myasthenia gravis.展开更多
BACKGROUND： The clinical values of neostigmine test, clinical electrophysiologic study and acetylcholine receptor antibody detection in diagnosing myasthenia gravis （MG） found newly are unclear in China.OBJECTIVE：...BACKGROUND： The clinical values of neostigmine test, clinical electrophysiologic study and acetylcholine receptor antibody detection in diagnosing myasthenia gravis （MG） found newly are unclear in China.OBJECTIVE： To investigate the reference value of common clinical diagnosis parameters in correctly diagnosing untreated MG found newly.DESIGN： Retrospective case analysis.SETTING： Department of Neurology, Beijing Hospital, Ministry of Health.PARTICIPANTS： Totally 156 outpatients with MG admitted to Department of Neurology, Beijing Hospital,Ministry of Health between January 1999 and December 2002. The involved patients, 72 males and 84 females, were aged 2 - 79 years. They were classified according to Osserman＇s criteria： ⅡA 72, ⅡB 76, Ⅲ3 and Ⅳ 5. They were all subjected to being inquired of disease history, neostigmine test, and acetylcholine receptor antibody detection, met the diagnosis criteria of Neuroimmunology Committee of China, and confirmed by clinical electrophysiologic detections; Informed consents were obtained from all the involved subjects.METHODS： ①After admission, every patient was intramuscularly injected with 1.5 mg neostigmine; If the patient was a child, the injection dose was decreased according to his/her age. If his/her score of any observation index after injection was improved ≥ 50% as compared with before injection , his positive index was Set as positive. Positive neostigmine test was set if there was one positive index. ②Repetitive nerve stimulation and single fiber electromyography were performed with Dantec Keypoint electromyogram （EMG） apparatus. ③Acetylcholine receptor antibody was detected by ELISA method.MAIN OUTCOME MEASURES： Clinical absolute and relative scores of MG, acetylcholine receptor antibody level, and repetitive nerve stimulation and single fiber electromyography examination results.RESULTS： The positive rates of neostigmine test, repetitive nerve stimulation and single fiber electromyography examination for MG were 86.5%, 82.6%, and 69.2%, respectively, and the positive rate of acetylcholine receptor antibody was 78.8%.CONCLUSION： Standardized neostigmine test has the highest sensitivity to diagnose MG.展开更多
Objective To evaluate the frequency, distribution and clinical significance of the antibodies to the fetal and/or adult acetylcholine receptor （AChR） in patients with myasthenia gravis （MG）. Methods AChR antibodie...Objective To evaluate the frequency, distribution and clinical significance of the antibodies to the fetal and/or adult acetylcholine receptor （AChR） in patients with myasthenia gravis （MG）. Methods AChR antibodies were detected by cell-based assay in the serum of ocular MG （OMG） （n = 90） and generalized MG （GMG） patients （n = 110）. The fetal-type （2α： β： γ： δ） and adult-type （2α： β： ε： δ） AChR were used as antigens, and their relevance to disease presentation was assessed. Results The overall frequencies of anti-adult and anti-fetal AChR antibodies were similar in all 200 patients examined, with 14 having serum specific to the AChR-γ subunit, and 22 to the AChR-ε subunit. The overall sensitivity when using the fetal and adult AChR antibodies was higher than that when using the fetal AChR antibody only （P = 0.015）. Compared with OMG patients, the mean age at disease onset and the positive ratio of antibodies to both isoforms of the AChR were significantly higher in patients who subsequently progressed to GMG. Older patients and patients with both anti-fetal and anti-adult AChR antibodies had a greater risk for developing generalized disease [odds ratio （OR）, 1.03; 95% confidence interval （CI）, 1.01–1.06 and OR, 5.09; 95% CI, 2.23–11.62]. Conclusion Using both fetal-and adult-type AChRs as the antigens may be more sensitive than using either subtype. Patients with serum specific to both isoforms are at a greater risk of progressing to GMG. Patients with disease onset at an advanced age appear to have a higher frequency of GMG conversion.展开更多
文摘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.
文摘Anti-acetylcholine receptor antibodies （AAR） are considered pathognomonic and pathogenetic for myasthenia gravis （MG）. AAR detection confirms clinical diagnosis of MG. However, AAR is rarely detected in patients without MG. The underlying pathophysiological mechanisms in a normal subject without MG have not been adequately addressed in previous studies. The present study reports on a case study of a healthy, elderly man with high AAR titers for 14 years. Pathophysiological mechanisms could be due to AAR heterogeneity in specificity, affinity, and multiform, and to muscle variability in response to AAR.
文摘Anti-bungarotoxin anti-serum,which has the internal image of nicotinicacetylcholine receptor,was used as a tool to measure anti-idiotypic antibodies toantibodies to Iigand of nicotinic acctylcholine receptor in scra from 81 patients withmyasthenia gravis.Enzyme-linked immunosorbcnt assay was adopted.Thc positive ratewas 46.9%(38/81).The specific cross inhibitory test with nicotinic acetylcholinereceptor was positive.Anti-idiotype antibodies to antibodies to ligand of nicotinicacetylcholine receptor in sera of different types of myasthenia gravis patients classified ac-cording to modified Osserman’s standard and myasthenia gravis patients with or withoutthymoma were comparcd in this study and the role of anti-idiotype antibodies toantibodies to Iigand of nicotinic acctylcholinc receptor in the immunity of myasthcniagravis and the possibility of thcrapeutic use of anti-idiotype antibodies arc discussed.
文摘The antibodies against acetylcholine receptor AchR and levels of SOD and LPO were measured in 11 patients with myasthenia gravis (MG),and the results were compared with normal controls and patients with diseases other than MG.The results showed that the antibodies against AchR were higher as compared with other groups before and after operation. The post-operative level of antibodies was obviously lower than the pre-operative value. An slight increase in SOD and significant decrease in mean value of LPO after surgery were noted. The possible mechanism was discussed.
基金supported by the National Natural Science Foundation of China,No.30360100,30760234,30860260,81160373,81360458
文摘Single-chain variable domain fragment （scFv） 637 is an antigen-specific scFv of myasthenia gravis. In this study, scFv and human serum albumin genes were conjugated and the fusion pro-tein was expressed in Pichia pastoris. The afifnity of scFv-human serum albumin fusion protein to bind to acetylcholine receptor at the neuromuscular junction of human intercostal muscles was detected by immunolfuorescence staining. The ability of the fusion protein to block myas-thenia gravis patient sera binding to acetylcholine receptors and its stability in healthy serum were measured by competitive ELISA. The results showed that the inhibition rate was 2.0-77.4%, and the stability of fusion protein in static healthy sera was about 3 days. This approach suggests the scFv-human serum albumin is a potential candidate for speciifc immunosuppressive therapy of myasthenia gravis.
文摘BACKGROUND： The clinical values of neostigmine test, clinical electrophysiologic study and acetylcholine receptor antibody detection in diagnosing myasthenia gravis （MG） found newly are unclear in China.OBJECTIVE： To investigate the reference value of common clinical diagnosis parameters in correctly diagnosing untreated MG found newly.DESIGN： Retrospective case analysis.SETTING： Department of Neurology, Beijing Hospital, Ministry of Health.PARTICIPANTS： Totally 156 outpatients with MG admitted to Department of Neurology, Beijing Hospital,Ministry of Health between January 1999 and December 2002. The involved patients, 72 males and 84 females, were aged 2 - 79 years. They were classified according to Osserman＇s criteria： ⅡA 72, ⅡB 76, Ⅲ3 and Ⅳ 5. They were all subjected to being inquired of disease history, neostigmine test, and acetylcholine receptor antibody detection, met the diagnosis criteria of Neuroimmunology Committee of China, and confirmed by clinical electrophysiologic detections; Informed consents were obtained from all the involved subjects.METHODS： ①After admission, every patient was intramuscularly injected with 1.5 mg neostigmine; If the patient was a child, the injection dose was decreased according to his/her age. If his/her score of any observation index after injection was improved ≥ 50% as compared with before injection , his positive index was Set as positive. Positive neostigmine test was set if there was one positive index. ②Repetitive nerve stimulation and single fiber electromyography were performed with Dantec Keypoint electromyogram （EMG） apparatus. ③Acetylcholine receptor antibody was detected by ELISA method.MAIN OUTCOME MEASURES： Clinical absolute and relative scores of MG, acetylcholine receptor antibody level, and repetitive nerve stimulation and single fiber electromyography examination results.RESULTS： The positive rates of neostigmine test, repetitive nerve stimulation and single fiber electromyography examination for MG were 86.5%, 82.6%, and 69.2%, respectively, and the positive rate of acetylcholine receptor antibody was 78.8%.CONCLUSION： Standardized neostigmine test has the highest sensitivity to diagnose MG.
基金supported by the National Natural Science Foundation of China (31170864 and 81171183)
文摘Objective To evaluate the frequency, distribution and clinical significance of the antibodies to the fetal and/or adult acetylcholine receptor （AChR） in patients with myasthenia gravis （MG）. Methods AChR antibodies were detected by cell-based assay in the serum of ocular MG （OMG） （n = 90） and generalized MG （GMG） patients （n = 110）. The fetal-type （2α： β： γ： δ） and adult-type （2α： β： ε： δ） AChR were used as antigens, and their relevance to disease presentation was assessed. Results The overall frequencies of anti-adult and anti-fetal AChR antibodies were similar in all 200 patients examined, with 14 having serum specific to the AChR-γ subunit, and 22 to the AChR-ε subunit. The overall sensitivity when using the fetal and adult AChR antibodies was higher than that when using the fetal AChR antibody only （P = 0.015）. Compared with OMG patients, the mean age at disease onset and the positive ratio of antibodies to both isoforms of the AChR were significantly higher in patients who subsequently progressed to GMG. Older patients and patients with both anti-fetal and anti-adult AChR antibodies had a greater risk for developing generalized disease [odds ratio （OR）, 1.03; 95% confidence interval （CI）, 1.01–1.06 and OR, 5.09; 95% CI, 2.23–11.62]. Conclusion Using both fetal-and adult-type AChRs as the antigens may be more sensitive than using either subtype. Patients with serum specific to both isoforms are at a greater risk of progressing to GMG. Patients with disease onset at an advanced age appear to have a higher frequency of GMG conversion.
