| Objective To study the clinical characteristics of bulbar myasthenia gravis (MG).Methods Retrospective study was done on 166 patients who were diagnosed at Tongji Hospital from May 1983 to October 2005.Results (1) Bulbar MG was a relatively rare type of MG, composed only 6.7% of all classifications. (2) The sex ratio was 1∶1.35 M∶F with the peak time of onset age at 20～40 years, there were more female patients than male ones. (3) There was a high morbidity of myasthenia crisis (26.5%). With fast exacerbations, myasthenia crisis occurred in 10.8% bulbar MG patients within six months after onset, and there was a high mortality rate (6.0%). (4) The patients were more liable to respiratory infections (18%), and the morbidity of pulmonary infection was higher (36.4%) when coincided by myasthenia gravis. (5) Incorrect diagnosis was common in bulbar MG (19.9%), which delayed prompt therapy, leading to poor efficacy. (6) The efficacy of routine therapy was not satisfactory. With corticosteroid therapy of moderate dose followed by small dose maintaining in 104 patients, remission was in 9.6%, improvement in 71.2% and 19.2% remained unchanged. Median dose cyclophosphamide therapy appeared to be an effective method for refractory MG in the near future. Thymectomy was performed in 25 patients, with positive efficacy in 80% in the long follow-up, and the total mortality rate was 12%.Conclusion The clinical data in this group suggested that bulbar MG had its distinct characteristics. The important information of the clinical manifestations available in this study may facilitate the diagnosis and therapy of bulbar MG. Objective The study was designed to evaluate the efficacy, safety, and using method Chinese herbs Tablet Fuzhengqiangjin therapy in treating the out-patients with myasthenia gravis (MG).Methods From February 2004 to March 2006, 164 ocular and gentle generalized myasthenic patients were classified randomly into three groups. Patients in group A were treated with prednisone and pyridostigmine, and those in group C were treated with the concoction of Chinese herbs Tablet Fuzhengqiangjin, and group B were treated with therapies both in group A and group C. Selected patients were followed up to study the efficacy, side-effects and using time point. The influences of sex, age at onset, the accompanying disorders and the level change of AchRAb on efficacy and the development of ocular MG had been analyzed. At the same time, the evolving pattern of ocular MG, and the role of short course of moderate-doses of daily prednisone therapy and followed by low-dose daily maintenance therapy on the evolution were observed.Results During a mean follow-up of nearly 1 years , the total effectiveness rate of Tablet Fuzhengqiangjin was only 46.9% , but it reduced significantly the relapse rate when the dose of prednisone tapered and the incidence of side-effects including Cushing′s reaction and respiratory tract infection. There were 15 ocular myasthenia patients developed into generalized myasthenia in 2 years. Both the efficacy of Tablet Fuzhengqiangjin and the symptom conversion of ocular myasthenia had no relations with sex, age at onset, the accompanying disorders and the level change of AchRAb. The long-term prednisone therapy reduced the evolution of ocular type into generalized myasthenia in 2 years.Conclusions (1) The concoction of Chinese herbs Tablet Fuzhengqiangjin made by ourselves might be helpful in controlling recurrence when prednisone therapy began to withdraw ,and could reduce the incidence of side-effects including Cushing′s reaction and respiratory tract infection. But, when administered alone, the efficacy was not so satisfactory. (2) The short course of moderate-doses of daily prednisone and followed by low–dose daily maintenance therapy can prevent the development of generalized disease in patients with ocular myasthenia gravis. (3) Further case-controlled studies and longer follow-up involving more patients treated with Tablet Fuzhengqiangjin are needed to evaluate the efficacy more exactly. |
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