大剂量丙种球蛋白、甲基强的松龙治疗重症肌无力

目的探讨依次采用大剂量丙种球蛋白静滴、大剂量的甲基强的松龙治疗重症肌无力的临床疗效。方法时符合Osserman分型的61例患者，随机分为两组：治疗组（A组）31例，依次采用大剂量丙种球蛋白厦大剂量的甲基强的松龙治疗；对照组（B组）30例，采用传统大剂量地塞米松静滴治疗；观察两组的临床疗效。结果治疗组症状缓解快，激素治疗过程中发生呼吸肌瘫痪、使用呼吸机现象少，住院时间短，疗效好（均P〈0．05）。结论依次采用大剂量丙种球蛋白、大剂量的甲基强的松龙冲击治疗重症肌无力的临床疗效好，值得临床推广。     

糖皮质激素治疗重症肌无力早期致病情加重的临床观察

目的观察甲基强的松龙冲击疗法(MPPT)治疗重症肌无力(MG)早期导致一过性肌无力加重的作用.方法对MPPT治疗的36例MG患者于治疗前及治疗后肌无力加重时分别进行临床评分、低频重复电刺激、血清AChRAb滴度检查.结果MPPT治疗MG后,1周时临床显效率58%,4周时临床显效率为78%,有效率为94%;部分患者出现一过性肌无力加重;加重后低频重复电刺激波幅递减幅度较加重前明显增加(P<0.05),而血清AChRAb滴度无明显变化(P>0.05).结论MPPT治疗MG,56%患者早期可出现不同程度的一过性肌无力加重,其中轻～中度加重占65%,重度加重占35%,14%累及呼吸肌,6%出现MG危象;加重多出现于治疗后的1～7(3±2)d,持续时间1～18(4±3)d;早期一过性肌无力加重可能与激素直接抑制神经-肌接头处传递有关.     

大剂量甲基强的松龙冲击治疗多发性硬化18例临床分析

目的 观察大剂量甲基强的松龙冲击治疗多发性硬化患者的疗效.方法 将36例多发性硬化患者随机分为2组,治疗组18例,使用甲基强的松龙1 g/d冲击治疗5d后改为口服强的松60mg/d,逐渐减量；对照组18例,使用地塞米松10mg/d,14 d后改为口服强的松60 mg/d,逐渐减量,2组连续治疗4周,观察2组疗效,比较有...     

隔日顿服强的松治疗重症肌无力疗效及安全性观察

目的 探讨隔日顿服强的松治疗重症肌无力（MG）患者的临床疗效及安全性。方法 对116例MG患者的临床及副作用表现进行跟踪观察。结果 初诊MG患者隔日顿服渐增法，显效率91．4％；MG危象患者，危象控制后强的松渐减法，均取得成功。未发现肌无力加重现象，副作用发生率为16．4％。结论 强的松可作为治疗MG的第一线药物，采用隔日顿服法临床疗效明显，避免初期肌无力加重现象，减少副作用发生。     

甲基强的松龙冲击联合鞘内注射地塞米松及甲氨蝶呤治疗狼疮脑病疗效观察

目的探讨甲基强的松龙冲击联合鞘内注射地塞米松及甲氨蝶呤治疗狼疮脑病的疗效。方法对16例狼疮脑病患者应用500 mg甲基强的松龙静滴,连续3 d,同时甲氨喋呤及地塞米松各5 mg鞘内注射,1周1~2次,观察临床症状及脑脊液指标。结果缓解13例,有效2例,死亡1例。结论甲基强的松龙冲击联合鞘内注射地塞米松及甲氨蝶呤是治疗狼疮脑病较安全有效的方法。     

中剂量环磷酰胺联合甲基强的松龙治疗重症肌无力危象探讨

目的 探讨中剂量环磷酰胺联合甲基强的松龙治疗重症肌无力危象的作用.方法 MG危象患者共41例,其中CTX组21例,对照组20例.CTX组予甲基强的松龙500 mg/d,连用3 d,后每隔3 d剂量减半.同时加用环磷酰胺0.4 g 静脉注射,连用3 d后改为0.2 g,隔天一次,直至呼吸困难消失,脱离呼吸机.对照组单独应...     

甲基强的松龙冲击疗法治疗儿童眼肌型重症肌无力临床研究

甲基强的松龙冲击疗法治疗多发性硬化和格林一巴利综合症已有少量报迫，治疗儿童眼肌型重症肌无力国内尚未见报告，我院用冲击疗法治疗25例取得显著效果，现报告如下：临床资料：本组25例．男11例，女14例，年龄4～11岁．平均年龄6．5岁．其病程均在2年以上，有9例经口服强的松3个月以上疗效欠佳。临床表现为单眼睑下垂的ZG例，双眼睑下垂4例，双眼睑交替下垂的1例。其中22倒出现程度不同的复机。25例经胸腺CT检查未发现胸腺癌，新斯的明试验和面神经重复频率刺激均为阳性。治疗方法：甲基强的松龙400～500mg或30mg／kg／日加入生理盐水3O…     

免疫冲击加机械通气抢救重症肌无力危象的研究

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。     

重症肌无力合并卵巢早衰一例

临床资料患者女，20岁，2005年7月出现双睑下垂，视物模糊，并逐渐出现说话费力，四肢乏力，症状有晨轻暮重、疲劳后加重、休息后好转的特点。同时伴有停经。2005年10月于外院确诊为“重症肌无力（MG）”，随后行性激素检查示卵泡刺激素（FSH）86．79IU／L，黄体生成素（LH）117．89IU／L，雌二醇（E2）33．45pg／ml，孕酮（PROG）0．29ng／ml，催乳素（PRL）15．06ng／ml，诊断为“卵巢性闭经”。于11月21日给予雌激素人工周期治疗，因服用妊马雌酮0．625g／d连续7d后感无力症状明显加重而停用。11月30日无其他诱因发生肌无力危象，给予吡啶斯的明和甲基泼尼松龙冲击治疗后肌无力症状缓解。12月下旬有1次少量月经来潮。2006年1月5日感冒后诱发第2次肌无力危象。     

硫唑嘌呤合用皮质类固醇治疗重症肌无力危象的疗效观察

目的:硫唑嘌呤合用皮质类固醇治疗重症肌无力危象的疗效观察。材料和方法:男女各3例。年龄26～48岁。其中5例重症肌无力眼肌型,1例重症肌无力延髓型。病程1.5～8年。都经胸腺瘤切除,术后2～5月出现重症肌无力危象。当皮质类固醇激素治疗效果不佳时,加用硫唑嘌呤100～150mg/天治疗。结果:在3～4周内完全脱离人工呼吸机,除轻度眼睑下垂,2例肢体肌力Ⅴ度、4例肢体肌力Ⅳ度。结论:硫唑嘌呤合用皮质类固醇治疗重症肌无力危象是有效的。     

Treatment of myasthenia gravis with high-dose intravenous immunoglobulin

We treated 37 patients affected by autoimmune generalized myasthenia gravis (MG) with high-dose intravenous gammaglobulin (HDIVIg), 400 mg/kg per day on 5 consecutive days. A one-degree improvement of Oosterhuis global clinical classification of myasthenic severity (OGCCMS), the disappearance of bulbar involvement or both were recorded 12 days after the beginning of the treatment in 70.3% of the patients and persisted up to 60 days in 58.7%. A two-degree improvement of OGCCMS was recorded in 54.1% of the patients and it was maintained up to 60 days in 37.8%. The percentage of improvement did not significantly differ between patients entering the treatment in a long-standing, drug-refractory stationary phase of the illness (n = 26) and patients who received HDIVIg in an acute phase of MG (n = 11). None of the patients experienced side effects. Our data indicates that HDIVIg is an interesting, virtually riskless therapeutic choice for MG patients, and allows the planning of a controlled trial versus plasma-exchange.     

Efficacy of low-dose FK506 in the treatment of Myasthenia gravis--a randomized pilot study

To determine the efficacy of low-dose FK506 in the treatment of myasthenia gravis (MG), untreated de novo patients were randomly selected to receive treatment with (n = 18) or without (n = 16) FK506, and were evaluated for 1 year after treatment with limitation of daily dose of prednisolone. Low-dose FK506 reduced the duration of early-phase therapy in hospital (p < 0.05) and the need for combined therapy with plasmapheresis and high-dose intravenous methylprednisolone or high-dose intravenous methylprednisolone alone (p < 0.05). It also reduced the daily dose of prednisolone (p < 0.05) required to maintain minimal manifestations of MGFA postintervention status. None of the patients exhibited significant side effects up to 1 year after treatment. These findings suggest that low-dose FK506 is safe and efficacious for the treatment of de novo MG patients.     

Immunoglobulin treatment in refractory Myasthenia gravis

Failure to induce and maintain remission in severe exacerbations of myasthenia gravis (MG), despite optimal care, is a common problem. We evaluated the efficacy and safety of high-dose intravenous immunoglobulin (IVIg) therapy in an open-label study of 10 patients with severe generalized myasthenia and an acute deterioration unresponsive to conventional therapy including high-dose corticosteroids, cyclosporine, and azathioprine. Intravenous Ig at a loading dose of 400 mg/kg was administered daily for 5 consecutive days, with maintenance IVIg treatment at a dose of 400 mg/kg, once every 6 weeks. Significant improvement occurred in all patients, beginning at 6 +/- 2 days of treatment as measured by the Osserman scale, fatigue variables, muscle strength, and respiratory function tests. No side effects were observed during induction of remission. Further IVIg treatments were highly efficacious in maintaining the remission. The severity of the disease decreased by 2.5 +/- 0.8 grades of the Osserman scale over a period of 1 year (P <0.001), in parallel with reduction of immunosuppressive therapy as well as a decrease in acetylcholine receptor antibody titers (P < 0.01). Intravenous Ig therapy seems to be highly potent for inducing rapid improvement in refractory myasthenia during acute deterioration as well as for maintaining remission.     

营养支持对重症肌无力患者呼吸功能的影响

目的探讨营养支持在治疗重症肌无力患者中的应用价值。方法对78例MG患者常规治疗基础上给予肠内营养乳剂进行营养支持15d，记录患者肠内营养治疗前后营养指标（肱三头肌皮皱厚度、上臂肌困、白蛋白、肌酐、尿素氮、氮平衡）、呼吸功能指标（最大吸气压、最大呼气压、残气量、最大通气量、呼吸肌耐力）及临床肌无力严重度的变化情况。将治疗前后的上述指标进行分析比较。结果MG患者肠内营养支持治疗15d后，患者各项营养指标中，血浆白蛋白、肌酐水平和氮平衡均较营养支持前明显升高（P〈0．01），血尿素氮（BUN）水平较营养支持前明显下降（P〈0．01）；I型MG患者的肺功能状况已经较健康对照有所下降；经常规治疗和营养支持后，各型MG患者的肺功能状况以及临床肌无力严重度均有所改善。结论对MG患者，合理的肠内营养支持治疗可以改善机体的营养状态，改善患者肺功能及临床症状。     

Treatment of refractory myasthenia: "rebooting" with high-dose cyclophosphamide

Patients with myasthenia gravis (MG) who do not respond to conventional immunotherapeutic agents, or cannot tolerate their side effects, are considered "refractory." Ablation of the immune system followed by bone marrow transplant has been shown to cure experimental MG in rats. It is now known that immunoablative treatment with high-dose cyclophosphamide does not damage hematopoietic "stem cells," permitting repopulation of the immune system without bone marrow transplant. Recent evidence indicates that this treatment can induce durable remissions in autoimmune diseases. We treated three myasthenic patients, for whom treatment with thymectomy, plasmapheresis, and conventional immunotherapeutic agents failed, by using high-dose cyclophosphamide (50mg/kg/day intravenously for 4 days) followed by granulocyte colony stimulating factor. All three patients tolerated the treatment well and have had marked improvement in myasthenic weakness, permitting reduction of immunosuppressive medication to minimal levels. Acetylcholine receptor (AChR) antibody levels decreased in two AChR antibody-positive patients, and anti-MuSK antibody levels decreased in one "AChR antibody-negative" patient. The patients have been followed for up to 3.5 years, with no recurrence of symptoms. High-dose cyclophosphamide treatment appears to be an effective and safe treatment for selected patients with refractory MG. Further follow-up of these and additional patients will be needed to determine whether the benefit is durable.     

胸腺放射治疗重症肌无力的疗效观察

目的 观察胸腺放射治疗重症肌无力（MG）的疗效。方法 回顾性分析我院自1957年到1999年42年间应用胸腺放射治疗的68例MG患者（深部X线放疗22例，^60Co治疗46例）的临床资料，并对他们作了长期随访。结果 放射治疗后总缓解率达90%，临床疗效显著，明显改善了患者生活质量，副反应以皮肤损害（10例）和消化道症状（9例）多见。结论 对于药物反应差，手术时扫现胸腺瘤对周围组织有明显浸润或术后症状不稳定MG患者加用胸腺放射治疗，可明显提高疗效。     

Single fiber EMG as an outcome measure in myasthenia gravis: results from a double-blind, placebo-controlled trial.

In a placebo-controlled, therapeutic, pilot trial of mycophenolate mofetil (MM) in autoimmune myasthenia gravis (MG), the authors compared pretreatment and posttreatment single fiber electromyography (SFEMG) jitter measurements performed on the same muscle in a total of 11 patients. The mean jitter value decreased (improved) by an average of 15.4 micros in patients receiving MM (n = 6), compared to an increase (worsening) in mean jitter of 4.0 micros in patients receiving placebo (n = 5). This difference was statistically significant (P = 0.030). In most patients, the change in SFEMG measurements correlated with the change in clinical state as measured by quantitative testing of muscle function. The authors conclude that immunomodulation by MM improves neuromuscular junction function in MG and that SFEMG may be a useful marker of early response in future therapeutic trials in autoimmune MG.     

免疫抑制剂对重症肌无力病人T淋巴细胞亚型的影响

目的观察重症肌无力(MG)病人细胞免疫的异常改变和免疫抑制剂对重症肌无力(MG)病人的临床疗效及对T淋巴细胞亚型的影响。方法用许氏评分法观察60例MG病人的病情严重程度和免疫抑制治疗2个月后的临床疗效;采用直接免疫荧光染色和流式细胞仪技术测定60例病人和60名志愿健康者周围血中T淋巴细胞亚型的百分率,并测定糖皮质激素或环磷酰胺免疫抑制治疗前及治疗后2个月T淋巴细胞亚型的变化。结果未治疗MG组外周血中CD8 T淋巴细胞的百分率较正常对照组显著下降(P<0.01);CD4 T淋巴细胞的百分率和CD4 /CD8 T细胞的比例较正常对照组均显著升高(P<0.01)。经激素或环磷酰胺治疗2个月后MG组随着临床症状的改善,外周血中总T细胞(CD3 )和CD8的百分率较治疗前均显著升高(P<0.01;P<0.05);CD4 T淋巴细胞的百分率和CD4 /CD8 T细胞的比例较治疗前均显著下降(P<0.01)。结论MG病人有T淋巴细胞亚型的变化,免疫抑制治疗对T淋巴细胞亚群有明显影响,提示T淋巴细胞亚型的测定可为激素、环磷酰胺等免疫疗法提供一个客观的实验室指标,为判断疾病的转归提供实验依据。     

重症肌无力合并多发性肌炎两例临床分析并文献复习

目的观察重症肌无力(MG)合并多发性肌炎(PM)的临床特征及疗效,并探讨其共病机制及治疗方法,提高临床识别度。方法报道作者医院收治的两例MG合并PM患者的临床资料,结合文献分析其发病特点、实验室检查、疗效及预后等。结果两例患者临床症状以四肢无力、球肌麻痹、肌肉疼痛为主要表现,疲劳现象不明显,实验室检查可见肌酶升高,偶有心肌受累,肌电图提示肌源性损害;单用溴吡斯的明治疗效果不佳,联合激素和免疫制剂治疗反应良好。结论 MG合并PM的病例少见;MG患者在规范化治疗过程中若出现肌肉疼痛、肌酶谱升高,或疲劳现象消失、眼肌症状不明显等表现时,需行肌电图、肌活检等明确有无合并肌肉疾病的可能;治疗上以免疫治疗为主,急重症者建议使用丙种球蛋白或血浆置换等方法。