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1.
Pediatr Neurol ; 156: 198-207, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-38810600

RESUMO

BACKGROUND: This study evaluated the efficacy and safety of eculizumab, a terminal complement C5 inhibitor, in juvenile generalized myasthenia gravis (gMG). METHODS: Adolescents aged 12 to 17 years with refractory anti-acetylcholine receptor (AChR) antibody-positive gMG received eculizumab (weekly induction [one to two doses of 600 mg or four doses of 900 mg] followed by maintenance doses [300 to 1200 mg] every two weeks for up to 26 weeks) in a phase 3, open-label multicenter study (NCT03759366). Change from baseline to week 26 in Quantitative Myasthenia Gravis (QMG) total score (primary end point) and secondary end points including Myasthenia Gravis-Activities of Daily Living (MG-ADL) total score, Myasthenia Gravis Composite score, Myasthenia Gravis Foundation of America postintervention status, EuroQol 5-Dimensions (Youth) and Neurological Quality-of-Life Pediatric Fatigue questionnaire scores, as well as pharmacokinetics, pharmacodynamics, and safety, were recorded. RESULTS: Eleven adolescents (mean ± S.D. age 14.8 ± 1.8 years) were enrolled; 10 completed the primary evaluation period. Least-squares mean changes from baseline at week 26 were -5.8 (standard error [SE] 1.2; P = 0.0004) for QMG total score and -2.3 (SE 0.6; P = 0.0017) for MG-ADL total score. Overall, the primary and all secondary efficacy end point analyses met statistical significance from the first assessment and were sustained throughout. Complete terminal complement inhibition was sustained through 26 weeks in all patients. Treatment-emergent adverse events were all mild/moderate and predominantly unrelated to eculizumab. CONCLUSIONS: Eculizumab was effective in reducing disease burden and was well tolerated in adolescents with refractory AChR antibody-positive gMG.


Assuntos
Anticorpos Monoclonais Humanizados , Inativadores do Complemento , Miastenia Gravis , Humanos , Adolescente , Anticorpos Monoclonais Humanizados/administração & dosagem , Anticorpos Monoclonais Humanizados/efeitos adversos , Anticorpos Monoclonais Humanizados/farmacologia , Miastenia Gravis/tratamento farmacológico , Masculino , Feminino , Criança , Inativadores do Complemento/administração & dosagem , Inativadores do Complemento/farmacologia , Resultado do Tratamento , Qualidade de Vida , Avaliação de Resultados em Cuidados de Saúde
2.
Adv Ther ; 41(6): 2486-2499, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38642198

RESUMO

INTRODUCTION: Efgartigimod and ravulizumab, both approved for treating acetylcholine receptor auto-antibody-positive (AChR-Ab+) generalized myasthenia gravis (gMG), have not been directly compared. This paper assessed comparative effects of efgartigimod vs. ravulizumab for treating adults with AChR-Ab+ gMG using indirect treatment comparison methods. METHODS: The matching-adjusted indirect comparison used data from two randomized trials of adult men and women. The ADAPT (efgartigimod vs. placebo; individual patient data available) population was reweighted to match the CHAMPION (ravulizumab vs. placebo; index study; aggregate data available) population. The relative effect of efgartigimod versus placebo was estimated in this reweighted population and compared with the observed ravulizumab versus placebo effect to estimate the efgartigimod versus ravulizumab effect. The outcomes were Myasthenia Gravis Activities of Daily Living (MG-ADL), Quantitative Myasthenia Gravis (QMG), and Myasthenia Gravis Quality of Life 15-item-revised scale (MG-QoL15r) assessed as cumulative effect (area under the curve; AUC) over 26 weeks (primary) and change from baseline at 4 weeks and time of best response (week 4 for efgartigimod; week 26 for ravulizumab). RESULTS: For MG-QoL15r, efgartigimod had a statistically significant improvement compared with ravulizumab over 26 weeks [mean difference (95% confidence interval): - 52.6 (- 103.0, - 2.3)], at week 4 [- 4.0 (- 6.6, - 1.4)], and at time of best response [- 3.9 (- 6.5, - 1.3)]. Efgartigimod had a statistically significant improvement over ravulizumab in MG-ADL at week 4 [- 1.9 (- 3.3, - 0.5)] and at time of best response [- 1.4 (- 2.8, 0.0)] and in QMG at week 4 [- 3.2 (- 5.2, - 1.2)] and at time of best response [- 3.0 (- 5.0, - 1.0)]. For AUC over 26 weeks, improvements were not significantly different between efgartigimod and ravulizumab for MG-ADL [- 8.7 (- 36.1, 18.8)] and QMG [- 13.7 (- 50.3, 22.9)]. CONCLUSION: Efgartigimod may provide a faster and greater improvement over 26 weeks in quality of life than ravulizumab in adults with AChR-Ab+ gMG. Efgartigimod showed faster improvements in MG-ADL and QMG than ravulizumab.


Assuntos
Anticorpos Monoclonais Humanizados , Miastenia Gravis , Receptores Colinérgicos , Humanos , Miastenia Gravis/tratamento farmacológico , Masculino , Feminino , Anticorpos Monoclonais Humanizados/uso terapêutico , Pessoa de Meia-Idade , Receptores Colinérgicos/imunologia , Adulto , Autoanticorpos , Resultado do Tratamento , Idoso , Atividades Cotidianas , Qualidade de Vida
3.
Neurol Sci ; 45(4): 1707-1717, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-37940750

RESUMO

BACKGROUND AND OBJECTIVES: Myasthenia gravis (MG) is an autoimmune disease affecting the neuromuscular junction. No cohort study has investigated the efficacy of inactivated vaccines in patients with MG. MATERIALS AND METHODS: This prospective observational cohort study included healthy controls (HCs) and patients with MG with or without immunosuppressive treatment. Vaccination occurred between May and December 2021. Patients with MG were subjected to a clinical scale assessment for disease severity. The neutralization antibodies (Nabs) levels were measured in all participants using the pseudovirus neutralization assay. RESULTS: Twenty-one patients (Female/Male:10/11); age median [interquartile range (IQR)]: 43 [30, 56]) were included in this study. Two patients (2/21) were lost during follow-up after enrollment. No sustained vaccine-related adverse effects occurred in any visit of patients with MG. No exacerbation of MG was observed. Acetylcholine receptor antibody (AChR-Ab) levels showed no statistically significant changes between the first and second visit (median [IQR]: 2.22 [0.99, 2.63] nmol/L vs. 1.54 [1.07, 2.40] nmol/L, p = 0.424). However, levels of AChR-Ab decreased at the third visit (median [IQR]: 2.22 [0.96, 2.70] nmol/L vs. 1.69 [0.70, 1.85] nmol/L, p = 0.011). No statistically significant difference in Nabs levels was found between HCs and patients with MG (median [IQR]: 102.89 [33.13, 293.86] vs. 79.29 [37.50, 141.93], p = 0.147). DISCUSSION: The safety of the SARS-CoV-2 inactivated vaccine was reconfirmed in this study. No significant difference in Nabs level was found between patients with MG and HCs. Nabs levels correlated with AChR-Ab levels before vaccination and ΔAChR-Ab levels.


Assuntos
COVID-19 , Miastenia Gravis , Adulto , Feminino , Humanos , Masculino , Estudos de Coortes , Vacinas contra COVID-19/efeitos adversos , Miastenia Gravis/tratamento farmacológico , Estudos Prospectivos , SARS-CoV-2 , Vacinas de Produtos Inativados/efeitos adversos , Pessoa de Meia-Idade
4.
Front Immunol ; 14: 1270011, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-38124751

RESUMO

Multi-antibody-positive myasthenia gravis (MG) presentations are relatively rare, often found in older patients, and generally predict a poor prognosis. We report a case of a female patient with generalized MG, testing positive for Titin antibodies (Titin-Ab), ryanodine receptor antibodies (RyR-Ab), and acetylcholine receptor antibodies (AChR-Ab), and resistant to acetylcholinesterase inhibitors. Following unsuccessful traditional therapies, she received Telitacicept, leading to significant improvements. This case underscores Telitacicept's potential efficacy for similar patients and offers insights into the clinical characteristics of multi-antibody MG.


Assuntos
Miastenia Gravis , Timoma , Neoplasias do Timo , Humanos , Feminino , Idoso , Conectina , Acetilcolinesterase , Miastenia Gravis/diagnóstico , Miastenia Gravis/tratamento farmacológico , Autoanticorpos
5.
JACC Case Rep ; 9: 101734, 2023 Mar 01.
Artigo em Inglês | MEDLINE | ID: mdl-36909266

RESUMO

Anti-Kv1.4 antibody is often detected in thymoma-associated myasthenia gravis patients with anti-acetylcholine receptor antibody. Herein, we describe 2 patients with concurrent myocarditis and myositis. In both cases, anti-Kv1.4 antibody was positive despite the absence of thymoma and anti-acetylcholine receptor antibody, and immunosuppressants eventually resolved their symptoms and cardiac function. (Level of Difficulty: Advanced.).

6.
Front Oncol ; 13: 1061264, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-36845745

RESUMO

Introduction: According to the principle, thymomas combined with myasthenia gravis (MG) require surgical treatment. However, patients with non-MG thymoma rarely develop MG and early- or late-onset MG after surgery is called postoperative MG (PMG). Our study used a meta-analysis to examine the incidence of PMG and risk factors. Methods: Relevant studies were searched for in the PubMed, EMBASE, Web of Science, CNKI,and Wanfang databases. Investigations that directly or indirectly analyzed the risk factors for PMG development in patients with non-MG thymoma were included in this study. Furthermore, risk ratios (RR) with 95% confidence intervals (CI) were pooled using meta-analysis, and fixed-effects or random-effects models were used depending on the heterogeneity of the included studies. Results: Thirteen cohorts containing 2,448 patients that met the inclusion criteria were included. Metaanalysis revealed that the incidence of PMG in preoperative patients with non-MG thymoma was 8%. Preoperative seropositive acetylcholine receptor antibody (AChR-Ab) (RR = 5.53, 95% CI 2.36 - 12.96, P<0.001), open thymectomy (RR =1.84, 95% CI 1.39 - 2.43, P<0.001), non-R0 resection (RR = 1.87, 95% CI 1.36 - 2.54, P<0.001), world health organization (WHO) type B (RR =1.80, 95% CI 1.07 - 3.04, P= 0.028), and postoperative inflammation (RR = 1.63, 95% CI 1.26 - 2.12, P<0.001) were the risk factors for PMG in patients with thymoma. Masaoka stage (P = 0.151) and sex (P = 0.777) were not significantly associated with PMG. Discussion: Patients with thymoma but without MG had a high probability of developing PMG. Although the incidence of PMG was very low, thymectomy could not completely prevent the occurrence of MG. Preoperative seropositive AChR-Ab level, open thymectomy, non-R0 resection, WHO type B, and postoperative inflammation were risk factors for PMG. Systematic Review Registration: https://www.crd.york.ac.uk/PROSPERO/, identifier CRD42022360002.

7.
Biotechnol Genet Eng Rev ; : 1-9, 2023 Jan 14.
Artigo em Inglês | MEDLINE | ID: mdl-36641594

RESUMO

To probe into the changes and significance of serum acetylcholine receptor antibody (AchR-Ab) and citric acid extractive antibody (CAE-Ab) in patients with thymoma after thoracoscopic surgery (TS). The data of 50 patients with thymoma receiving TS in our hospital from February 2017 to February 2021 were selected for retrospective analysis. Serological testing was performed before and after surgery to determine serum AchR-Ab and CAE-Ab levels, the therapeutic effect was evaluated and the trend of serum AchR-Ab and CAE-Ab changes and their meanings were analyzed. Among 50 patients with thymoma after TS, 15 (30.0%) were in remission, 25 (50.0%) had improvement and 10 (20.0%) had no response to treatment. After treatment, the serum AchR-Ab and CAE-Ab levels of patients, which were remarkably lower than those before treatment (P < 0.001), were remarkably lower in the remission group than in the improvement group (P < 0.001) and remarkably lower in the improvement group than in the ineffective group (P < 0.001). The ordinal logistic regression analysis concluded that the levels of AchR-Ab and CAE-Ab were related to therapeutic effect, i.e. the lower the serum AchR-Ab and CAE-Ab levels, the better the therapeutic effect. TS can reduce the serum AchR-Ab and CAE-Ab levels in patients with thymoma, and serum AchR-Ab and CAE-Ab levels can reflect the therapeutic effect, providing reference value.

8.
Zhongguo Zhen Jiu ; 42(7): 755-9, 2022 Jul 12.
Artigo em Chinês | MEDLINE | ID: mdl-35793884

RESUMO

OBJECTIVE: To compare the clinical efficacy between acupuncture combined with western medication and simple western medication for ocular myasthenia gravis (OMG), and to explore its possible mechanism. METHODS: A total of 60 patients of ocular myasthenia gravis were randomized into an acupuncture combined with western medication group (30 cases, 1 case dropped off) and a western medication group (30 cases, 2 cases dropped off). Oral pyridostigmine bromide tablet and prednisone acetate tablet were given in the western medication group. On the basis of the treatment in the western medication group, Tongdu Tiaoqi acupuncture (acupuncture for unblocking the governor vessel and regulating qi ) was applied at Baihui (GV 20), Fengfu (GV 16), Hegu (LI 4), Zusanli (ST 36), etc. in the acupuncture combined with western medication group, once a day, 6 days a week. The treatment was given 8 weeks in both groups. Before and after treatment, the OMG clinical absolute score was observed, electrophysiological indexes of orbicularis oculi (value of mean jitter, percentage of jitter >55 µs and percentage of blocks) were measured by single-fiber electromyography (SFEMG), serum levels of acetylcholine receptor antibody (AChR-Ab), interferon-gamma (IFN-γ) and interleukin-4 (IL-4) were detected by ELISA method. RESULTS: After treatment, the OMG clinical absolute scores, values of mean jitter, percentages of jitter >55 µs, percentages of blocks and serum levels of AChR-Ab, IFN-γ and IL-4 were decreased compared before treatment in both groups (P<0.05), and those in the acupuncture combined with western medication group were lower than the western medication group (P<0.05). CONCLUSION: Acupuncture combined with western medication can effectively improve ptosis, palpebra superior fatigability, eye movement disorder and neuromuscular junction dysfunction in patients with ocular myasthenia gravis, the therapeutic effect is superior to simple western medication. Its mechanism may be related to down-regulating serum levels of AChR-Ab, IFN-γ and IL-4 and promoting the recovery of orbicularis oculi function.


Assuntos
Terapia por Acupuntura , Miastenia Gravis , Músculos Faciais , Humanos , Interferon gama , Interleucina-4 , Miastenia Gravis/tratamento farmacológico
9.
Artigo em Chinês | WPRIM (Pacífico Ocidental) | ID: wpr-939528

RESUMO

OBJECTIVE@#To compare the clinical efficacy between acupuncture combined with western medication and simple western medication for ocular myasthenia gravis (OMG), and to explore its possible mechanism.@*METHODS@#A total of 60 patients of ocular myasthenia gravis were randomized into an acupuncture combined with western medication group (30 cases, 1 case dropped off) and a western medication group (30 cases, 2 cases dropped off). Oral pyridostigmine bromide tablet and prednisone acetate tablet were given in the western medication group. On the basis of the treatment in the western medication group, Tongdu Tiaoqi acupuncture (acupuncture for unblocking the governor vessel and regulating qi ) was applied at Baihui (GV 20), Fengfu (GV 16), Hegu (LI 4), Zusanli (ST 36), etc. in the acupuncture combined with western medication group, once a day, 6 days a week. The treatment was given 8 weeks in both groups. Before and after treatment, the OMG clinical absolute score was observed, electrophysiological indexes of orbicularis oculi (value of mean jitter, percentage of jitter >55 μs and percentage of blocks) were measured by single-fiber electromyography (SFEMG), serum levels of acetylcholine receptor antibody (AChR-Ab), interferon-gamma (IFN-γ) and interleukin-4 (IL-4) were detected by ELISA method.@*RESULTS@#After treatment, the OMG clinical absolute scores, values of mean jitter, percentages of jitter >55 μs, percentages of blocks and serum levels of AChR-Ab, IFN-γ and IL-4 were decreased compared before treatment in both groups (P<0.05), and those in the acupuncture combined with western medication group were lower than the western medication group (P<0.05).@*CONCLUSION@#Acupuncture combined with western medication can effectively improve ptosis, palpebra superior fatigability, eye movement disorder and neuromuscular junction dysfunction in patients with ocular myasthenia gravis, the therapeutic effect is superior to simple western medication. Its mechanism may be related to down-regulating serum levels of AChR-Ab, IFN-γ and IL-4 and promoting the recovery of orbicularis oculi function.


Assuntos
Humanos , Terapia por Acupuntura , Músculos Faciais , Interferon gama , Interleucina-4 , Miastenia Gravis/tratamento farmacológico
10.
Gland Surg ; 10(8): 2408-2413, 2021 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-34527552

RESUMO

BACKGROUND: Approximately 15-29.6% of patients with thymoma have myasthenia gravis (MG). Some of these patients develop MG after thymectomy despite having no history of MG or related symptoms. Few previous studies have examined the risk factors for the development of post-thymectomy MG in patients with thymoma. Herein, we retrospectively reviewed our institutional experience with patients with thymoma who developed MG after thymectomy. METHODS: Twenty-six patients with thymoma but without MG, who were tested preoperatively for anti-acetylcholine receptor antibody (anti-AChR-Ab) levels, underwent surgical resection at our hospital between 2013 and 2020. Patients with thymic carcinoma were excluded from the study. We evaluated the association of outcomes with preoperative anti-AChR-Ab levels and post-thymectomy MG. We performed a χ2 test for bivariate analysis of categorical data. Differences were considered significant at P<0.05. RESULTS: The characteristics of the 26 patients (median age: 62 years; 8 men, 18 women) were as follows: World Health Organization (WHO) classifications AB (n=8), B1 (n=9), B2 (n=6), B3 (n=1), and others (n=2) and Masaoka stage I (n=12), II (n=9), III (n=3), and IVa (n=2). Among the 26 patients, only five had high (>0.3 nmol/L) preoperative anti-AChR-Ab levels. Post-thymectomy MG occurred in two of the five patients (40%) with high preoperative anti-AChR-Ab levels. A high preoperative serum anti-AChR-Ab titer was significantly associated with post-thymectomy MG (P=0.0267). The anti-AChR-Ab titer was also measured postoperatively in four of the five (80%) patients with high preoperative levels. The anti-AChR-Ab titer decreased in two of these four patients, and neither developed postoperative MG. CONCLUSIONS: Preoperative and postoperative anti-AChR-Ab positivity might be associated with post-thymectomy MG. Therefore, regular measurement of anti-AChR-Ab levels after thymectomy is required.

11.
Front Neurol ; 12: 755356, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34975721

RESUMO

Introduction: The changes in the number and function of regulatory T cells (Tregs) are thought to play important roles in the pathogenesis of generalized myasthenia gravis (gMG). Previous studies have suggested the decrease of FoxP3+ Treg cells in the MG development. However, there is no study on the pathophysiological mechanism of FoxP3-Treg, especially Tr1 cells, in gMG patients. Therefore, this study was conducted to reveal the effect of Tr1 cells to the pathophysiology of gMG. Methods: Thirteen patients with gMG and twelve healthy volunteers were enrolled in this study. The titer of anti-AChR Ab was measured by ELISA. The separated PBMCs were labeled for CD4, CD25, CD49b, LAG3 and FoxP3. The CD4+ T cell count, FoxP3+ Treg to CD4+ T cell ratio and Tr1 cell to CD4+ T cell ratio were measured by flow cytometry. Based on the FoxP3+ Treg and Tr1 cell to CD4+ T cell ratios, the patients' Tr1 cell to FoxP3+ Treg ratios were calculated. The IL-6, IL-7, IL-10, TGF-ß and IFN-γ concentration in the serum of MG patients and normal controls (NCs) were measured via ELISA. Results: We found a significantly positive correlation between the Tr1 cell/CD4+ T cell ratio and the anti-AChR Ab (r = 0.6889 ± 0.4414, p = 0.0401). Although there were no significant differences in the relationship between FoxP3+ Treg cells and anti-AChR Ab, a positive correlation between the Tr1 cell/FoxP3+ Treg cell ratio and the anti-AChR Ab (r = 0.7110 ± 0.4227, p = 0.0318) was observed. In addition, the Tr1 cell/CD4+ T cell ratio but not the proportion of FoxP3+ Tregs was positively correlated with IL-10 (p = 0.048). These results suggested that in the process of the immunomodulatory effect of Tr1 cells in patients with gMG, IL-10 and other cytokines may be involved, but the specific mechanism needs further study. Conclusion: This is the first study of the immunoregulatory mechanism of Tr1 cells in gMG. We conducted this study to elucidate the significance of Tr1 cells in the pathogenesis of MG. We believe that in patients with gMG, Tr1 cells may play an immunomodulatory role in counteracting AChR-related autoimmune responses. In this process, IL-10 and other immunomodulatory cytokines may be involved.

13.
J Thorac Dis ; 9(6): 1592-1597, 2017 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-28740673

RESUMO

BACKGROUND: Microscopic thymomas, defined as epithelial proliferations smaller than 1 mm in diameter, characteristically occur in patients with myasthenia gravis without macroscopic thymic epithelial tumors. However, some clinical and pathological aspects of this entity are still unclear. METHODS: This retrospective study includes five consecutive patients who had undergone extended thymectomy for myasthenia gravis at our institution from April 2007 to March 2016 and in whom microscopic thymomas were diagnosed by histopathological examination of the resected specimens. During the same period, we performed 32 extended transsternal thymothymectomies/thymectomies in patients with myasthenia gravis, including the above five cases. We here review 18 cases of microscopic thymoma, including our five cases and 13 previously reported cases. RESULTS: The incidence of previously undiagnosed microscopic thymoma in patients undergoing thymectomy for myasthenia gravis in our institution is 15.2%. Serum preoperative anti-acetylcholine receptor antibody (anti-AchR Ab) titers were abnormally high in all of our five cases h (74.4±53.3 nmol/L) and decreased significantly after surgery (11.7±13.5 nmol/L, P=0.037). We divided our cases into the following three groups: microscopic thymoma group (Group M), thymoma group (Group T) and non-thymic tumor group (Group N). The mean preoperative anti-AchR Ab titers of these groups were 74.4, 26.5, and 368 nmol/L, respectively. All these values decreased postoperatively. The mean anti-AchR Ab titer was significantly higher in Group M than in Group T (P=0.034). All five cases in Group M were found by post-operative pathological examination to have multifocal type A thymomas. CONCLUSIONS: Microscopic thymomas tend to be multifocal type A thymomas. Anti-AchR Ab titers decreased significantly in all groups. It is very important to both perform complete extended thymectomies in patients with myasthenia gravis and pathological examination of thin slices of thymic tissue to maximize detection of microscopic thymomas.

14.
Chinese Journal of Immunology ; (12): 1462-1466, 2016.
Artigo em Chinês | WPRIM (Pacífico Ocidental) | ID: wpr-504373

RESUMO

Objective:To investigate the immune mechanism of Shengxian decoction in experimental autoimmune myasthenia gravis(EAMG) rats. Methods:Lewis rats were immunized with the rat sequence 97-116 of the AChRαsubunit(Rα97-116) in CFA, 25 of which were successful. They were randomly divided into 5 groups:EAMG model group,prednisone group(5. 4 mg/kg),Shengxian decoction low, medium, high dose groups ( dosage 2. 6 g/kg, 5. 2 g/kg, 10. 4 g/kg ) . Clinical symptoms, weight, and the decrement percentage of RNS(5 Hz) were evaluated,and ELISA were adopted to determine the titers of AChR Ab,TGF-β,IFN-γ,IL-2,IL-4 and IL-17 in serum. Results:After molding,the percentage of decrement of RNS in each group noticeably increased by more than 10% in comparison with that in the CFA control group ( P<0. 01 or P<0. 05 ) . At the same time, they were also subjected to progressive decreasing weight and typical myasthenia symptoms,showing the successful molding. With medication,the decrement percentage of RNS of rats in the groups with low,medium and high dose of Shengxian decoction were all on obvious decline with alleviated weight decrease (P<0. 01),testifying to the symptom improvement. Compared with the CFA control group,the groups with low,medium and high dose of Shengxian decoction were coupled with decreasing AChR Ab content(P<0. 05),rising TGF-βlevel and reducing IFN-γ,IL-2,IL-4 and IL-17 level(P<0. 01 or P<0. 05). Conclusion: Shengxian decoction can turn the decrement percentage of RNS around,improve the progressive weight decrease in EAMG rats and increase the weight gains. By up-regulating the TGF-βlevel,lowering IFN-γ,IL-2,IL-4 and IL-17 level,preventing B cells from producing AChR Ab and reducing the content of AChR Ab in serum,it will soothe the damage of NMJ to AChR and cure EAMG.

15.
Iran J Neurol ; 10(1-2): 22-5, 2011.
Artigo em Inglês | MEDLINE | ID: mdl-24250839

RESUMO

BACKGROUND: Several concomitant disorders especially thyroid abnormalities have been reported in patients with myasthenia gravis (MG). We aimed to estimate the frequency and pattern of thyroid disorders in Iranian patients with MG. METHODS: All consecutive patients with MG referred to neurology clinic of Rasool-e-Akram Hospital during 2006-2007 were enrolled. All patients underwent clinical assessment of thyroid gland as well as thyroid function test. AChR Ab titer was measured as well. Nerve conduction study (NCS), Electromyography (EMG), and Repetitive Nerve Stimulation (RNS) was done by a same neurologist. The diagnosis of MG was made on the basis of clinical examinations, an edrophonium chloride test and electrophysiological studies. The diagnosis of thyroid disorders were based on clinical presentation as well as thyroid function tests. RESULTS: FIFTY EIGHT PATIENTS (MEAN AGE [SD]: 37.1 [16.9], range: 10-80; female: 65.5%) were enrolled in this 12-month study. Four patients (6.9%) had abnormal thyroid function tests (Hypothyroidism: 3 [5.2%]; 4 females; 3 with hypothyroidism and 1 with hyperthyroidism). The mean age (SD) in men and women were 41.4 (21.3) and 34.9 (13.8) years (P: N.S.), respectively. In addition, once the MG patients are younger than 50, female gender is dominant while they are more than fifty, male is the dominant gender. CONCLUSION: Our results show that Iranian patients with MG tend to be female and young. Before sixth decade of life, women are the most presenting patients thereafter, men are the predominant gender. About 7 percent of them may suffer from concomitant thyroid problem especially hypothyroidism.

16.
Artigo em Coreano | WPRIM (Pacífico Ocidental) | ID: wpr-96005

RESUMO

PURPOSE: This study was undertaken to evaluate the clinical usefulness of Tensilon test, repetitive nerve stimulation test(RNST), single fiber EMG(SFEMG) test and acetylcholine receptor antibody(AchR Ab) assay for making diagnosis of myasthenia gravis(MG). METHOD: These tests were performed in 21 MG patients which were classified into 11 ocular, 5 mild generalized, 4 moderate generalized, and 1 chronic severe MG. RESULT: The overall positivity of Tensilon test, SFEMG and AchR Ab was 95%, 87%, and 76% respectively. The overall positivity of RNST was 67%; 38% on flexor carpi ulnaris, 43% on adductor digiti quinti and 62% on orbicularis oculi muscles. The positivity of each test was higher in generalized MG group than in ocular MG group. But we could observe the statistically significant difference only in the RNST(p<0.05). CONCLUSION: Tensilon test showed the highest positivity in all MG groups. So we would like to recommend the Tensilon test for the diagnosis of MG at first, followed by RNST and AchR Ab assay, and SFEMG would be indicated to MG group which showed relatively low postivity in other tests.


Assuntos
Humanos , Acetilcolina , Diagnóstico , Edrofônio , Músculos , Miastenia Gravis
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