大剂量激素冲击联合小剂量血浆置换治疗重症肌无力的疗效

目的:观察大剂量激素冲击治疗以及联合小剂量血浆置换治疗重症肌无力的效果。方法;采用回顾性研究方法分析６７例患者用不同方法治疗的疗效,疗效判定用临床绝对评分和相对评分法,结果:各组治疗前后临床绝对评分有显著性差异,ｌ个月后,单用吡啶斯的明组、激素冲击治疗组、联合小剂量血浆置换组的相对评分分别为２７%、４８%、８１%。大剂量激素组、联合血浆置换组起效时间分别为２~３周、１~２周。结论:大剂量激素冲击治疗重症肌无力疗效肯定,如辅以小剂量血浆置换疗效更佳。     

血浆置换联合激素治疗重症神经系统脱髓鞘疾病

目的　评价血浆置换联合激素治疗重症吉兰 -巴雷综合征 ( Guillain-Barre syndrome,GBS)和多发性硬化( multiple sclerosis,MS)的临床疗效。方法　回顾性分析血浆置换联合激素治疗的 1 3例重症 GBS和 7例重症MS的临床转归及治疗后血中免疫球蛋白滴度变化。结果　患者呼吸肌麻痹于治疗后 5～ 1 4d缓解 ,2个月内临床表现缓解率分别为 GBS76.9% ,MS5 7.1 %。血中免疫球蛋白滴度明显减少 ( P <0 .0 1 )。结论　血浆置换联合激素疗法疗效肯定 ,可作为治疗急性重症神经脱髓鞘疾病首选方法。     

血浆置换、丙种球蛋白、甲基强的松龙治疗重症肌无力的疗效观察与护理

目的　探讨依次采用少量血浆置换、大剂量丙种球蛋白与大剂量甲基强的松龙治疗重症肌无力 (MG)Ⅱb或Ⅲ型的临床疗效及安全性。方法　对符合Osserman分型的Ⅱb或Ⅲ型的 3 7例MG患者 ,按住院先后顺序随机分为观察组 18例 ,依次采用少量血浆置换、大剂量丙种球蛋白、大剂量甲基强的松龙治疗。对照组 19例 ,大剂量地塞米松静滴。结果　观察组症状缓解快 ,激素治疗过程中发生呼吸肌瘫痪与使用呼吸机现象少 ,住院时间短、死亡率低 (均P <0 0 5 )。结论　依次采用少量血浆置换、大剂量丙种球蛋白与大剂量甲基强的松龙治疗Ⅱb或Ⅲ型MG疗效显著 ,安全性高。     

膜式血浆置换治疗重症吉兰-巴雷综合征疗效观察

目的 观察膜式血浆置换（PE）治疗重症吉兰-巴雷综合征（GBS）的疗效。方法 回顾性分析16例应用膜式血浆置换治疗的重症吉兰-巴雷综合征患者的临床资料,比较PE开始于病后≤7d及〉7d两组患者的临床疗效;比较PE结合有创通气及无创→有创通气两种通气方法对治疗结果的影响。结果 PE开始于病后≤7d组2周时临床疗效明显优于〉7d组（P〈0．05）,无创→有创通气组治疗效果明显好于直接有创通气组（P〈0．05）。结论 即早膜式血浆置换对重症GBS疗效显著,PE结合无创→有创通气可作为治疗重症GBS的首选治疗方案。     

甲泼尼龙冲击后泼尼松延续治疗重症肌无力（57例随访观察）

目的:甲泼尼龙冲击疗法(MPPT)治疗重症肌无力(MG)57例的近期疗效及1～3年随访观察。方法:MPPT和泼尼松治疗57例重症肌无力患者,按美国重症肌无力协会临床分型为Ⅰ型25例,Ⅱa型13例,Ⅱb型7例,Ⅲa型5例,Ⅲb型3例,Ⅳb型1例,Ⅴ型3例,均予以甲泼尼龙,成人1000mg·d-1,儿童20～30mg/(kg·d)溶于5%葡萄糖液内缓慢静脉滴注,3d为1疗程,每月1疗程,共3疗程。结果:近期疗效(半个月内)为:完全缓解21例(36.8%),部分缓解29例(50.8%),未改善7例(12.3%);随访1、2和3年时的完全缓解率分别为81.1%、88.4%、93.2%。结论:MPPT治疗MG1～3年缓解率较高。     

抗体阴性重症肌无力的短程血浆置换疗法初探

血浆置换(plasma exchange,PE)在重症肌无力(MG)上的疗效已有报道,但缺乏明确一致的置换方案。我们对12例MG患者进行了37人次的短程PE,各例均在10d内行3～4次置换术,术前和术后第7天按Mantegazza肌力评分表进行临床疗效评定,结果除1例外,均获得肯定疗效,仅1人次发生较严重的副反应。4例抗体阴性病人置换后均获显著疗效,提示抗体阴性MG血浆中存在某些未知的致病因子。短程PE可供选择作为MG危象的治疗方案。     

血浆置换治疗在重症肌无力患者中的应用--附57例临床疗效分析

目的确定血浆置换（ＰＰ）治疗重症肌无力（ＭＧ）的起效时间、疗效，评价其疗效是否与降低血中乙酰胆碱受体抗体（ＡＣｈＲＡｂ）有关。方法ＰＰ第１周隔日１次，共３次，以后每周１次。用健康人血浆及血浆代用品进行ＰＰ，一般每次２０００ｍＬ（即７０６代血浆５００ｍＬ，血浆１５００ｍＬ），置换当日及置换后每日于固定时间根据临床绝对记分法进行评分，同时每日晨取血查ＡＣｈＲＡｂ。结果痊愈、基本痊愈、显效、好转和无效分别为１、４、２３、２２和６例，总有效率８９．４７％。共有３５人、５２次在置换中出现不同程度副作用，包括口周麻木、四肢麻木、荨麻疹、寒战、发热和低血压等，经对症处理后好转。结论ＰＰ起效迅速，在肌无力危象抢救中有重要价值，但单独使用疗效不持久，应用得当可减少副作用。     

神经系统疾病患者在血浆置换治疗中的护理

血浆置换治疗是将患者血液引入血浆交换装置,将分离出的血浆弃去,并补回一定量的血浆,从此清除患者血浆中抗体,激活免疫反应的介质和免疫复合物,从而达到治疗目的.血浆置换能广泛应用于临床各科疾病,尤其对神经系统的自身免疫性疾病的治疗,效果更为明显.2007-01～2009-01,我们对35例神经系统疾病,主要是重症肌无力及危象、吉兰-巴雷综合征,采用一次膜式血浆置换加透析疗法,2～3次后临床症状与体征好转,取得满意效果,现报道如下.     

胸腔镜胸腺扩大切除治疗儿童重症肌无力临床观察

目的探讨胸腔镜胸腺扩大切除术治疗儿童重症肌无力的临床应用价值。方法胸腔镜胸腺扩大切除术治疗儿童重症肌无力12例,并随访6~12月评价疗效。结果所有患儿均顺利完成手术,无严重围手术期并发症;术后随访6~12月,完全缓解4例,好转5例,无变化3例。结论胸腔镜胸腺扩大切除术创伤小、恢复快,是治疗儿童重症肌无力的有效方法。     

74例重症肌无力危象的临床分析

目的 探讨重症肌无力危象患者的临床特点和预后。方法 回顾74 例第一次发生肌无 力危象的重症肌无力患者的一般资料，分析Osserman 分型、危象发生时间、危象前特点、危象持续时间， 评价重症肌无力危象治疗效果及预后。结果 伴胸腺瘤的重症肌无力危象患者45 例，占60.81%，非胸 腺瘤患者29 例，占39.19%。60.81%（45/74）的肌无力危象发生在重症肌无力起病的1 年之内，胸腺切除 术后6 个月内发生危象的比例为76.00%（38/50）。呼吸费力和吞咽费力（24/74）是出现危象前最显著的特 征，其次为胸腺手术后（11/74）、感染（9/74）、激素相关（7/74）。所有危象患者中Osserman 分型ⅡB 型所占 比例最高，为45.95%（34/74）。重症肌无力危象患者的插管时间为15（7，30）d，44.60%（33/74）的危象患者 需要丙种免疫球蛋白和（或）血浆置换联合甲泼尼龙冲击治疗。肌无力危象最常见的并发症为肺部感染 （32/74，43.24%），反复气管插管最严重的并发症为支气管扩张伴出血、气胸。随访2～10 年，17 例患者 出现再次或多次危象，死亡率为9.46%（7/74）。结论 伴胸腺瘤的重症肌无力患者较不伴胸腺瘤患者出 现肌无力危象的比例更高。重症肌无力危象治疗困难，需要多种免疫抑制剂联合治疗。胸腺切除后的 半年内，仍然是肌无力危象发生的高峰。肌无力危象并发症、反复危象、胸腺瘤转移是患者死亡的主要 原因。     

双重血浆置换与常规疗法治疗急性吉兰-巴雷综合征的随机对照研究

目的 比较双重血浆置换（double filtration plasmapheresis，DFPP）与常规疗法治疗急性吉兰一巴雷综合征（Guillain-Barre syndrome，GBS）的临床疗效。方法 将60例急性GBS患者随机分为DFPP组及常规治疗组，观察治疗前后神经功能改善状况，并检测治疗前后血液中免疫系列、补体系列及总蛋白水平。结果两组MRC（medical research council）评分于治疗1个月后、Hughes评分于治疗半年后比较差异有统计学意义，治疗后DF-PP组血液中免疫球蛋白IgG、IgA及补体C3明显低于常规治疗组，总蛋白无明显变化。结论 DFPP是治疗急性GBS较为有效的方法，可明显改善患者神经功能缺损状况，其机制可能与降低血液中免疫球蛋白及补体有关。     

Double filtration plasmapheresis benefits myasthenia gravis patients through an immunomodulatory action

Double filtration plasmapheresis (DFPP) is used to treat myasthenia gravis (MG). However, the definite mechanism is unclear. This study investigated whether DFPP improves MG through an immunomodulatory action. Thirty-five MG patients were randomly divided into two treatment groups: Group A (DFPP combined with oral methylprednisolone) and Group B (oral methylprednisolone alone). Their antibody levels, clinical scores, cytokine levels, and CD4⁺CD25^highFoxp3⁺ (regulatory T cell [Treg]) levels were then determined. Anti-titin antibody levels were significantly lower in Group A compared with Group B after treatment. The clinical remission rate in Group A was significantly higher than in Group B. The changes in cytokine levels (interleukin [IL]-2, IL-4, IL-10, and interferon-γ) in sera and the peripheral blood mononuclear cell culture supernatants did not significantly differ before and after the treatments in both groups (p < 0.05). The soluble intercellular adhesion molecule-1 (sICAM-1) levels were lower in Group A than in Group B (p < 0.05). MG patients exhibited a lower percentage of Treg cells than normal patients. DFPP combined with methylprednisolone treatment increased the Treg cell percentage more than treatment with methylprednisolone alone (p < 0.05). DFPP treatment more effectively lowers sICAM-1 and increases Treg cell expression, consequently benefiting MG patients.     

免疫吸附与双重血浆置换治疗急性Guillain-Barre综合征疗效及安全性的研究

目的比较免疫吸附(IA)与双重血浆置换(DFPP)治疗急性Gu illain-Barre综合征(GBS)的疗效及安全性。方法60例急性GBS患者随机分为IA组及DFPP组,分别采用IA及DFPP方法治疗;观察治疗后神经功能改善状况;检测治疗前后血液中免疫系列、补体系列及总蛋白水平。结果IA组与DFPP组神经功能缺损程度均随时间变化而改善;两组间Hughes评分差异无显著性;半年后IA组MRC病情评分显著优于DFPP组(P<0.05)。两组血液中的补体C3及免疫球蛋白IgG、IgA水平与治疗前相比显著降低(P<0.05~0.01);两组间清除免疫球蛋白及补体量差异无显著性。DFPP组并发症的发生率高于IA组,但差异无显著性。结论IA及DFPP均是治疗急性GBS有效的方法,IA疗效及安全性优于DFPP。     

Therapeutic efficacy of double filtration plasmapheresis in patients with anti-aquaporin-4 antibody-positive multiple sclerosis

Multiple sclerosis (MS) in Asian countries, including Japan, is classified into two types: conventional MS (C-MS), characterized mainly by cerebral lesions, and opticospinal MS (OS-MS) or neuromyelitis optica (NMO), characterized by selective involvement of the optic nerve and spinal cord. Recently, a serum immunoglobulin-G-antibody was discovered in patients with NMO that targets aquaporin-4 (AQP4). The existence of the anti-AQP4 antibody shows the pathogenetic role of humoral immune factors in OS-MS/NMO. We treated eight patients with anti-AQP4 antibody-positive MS with double filtration plasmapheresis (DFPP) to remove the antibody. Improvement of vision was observed in two patients. Motion improvement was seen in seven patients. Sensory improvement was observed in four patients. In total, six out of eight patients (75%) showed therapeutic improvement after DFPP treatment. We propose that DFPP might be an effective therapeutic option for patients with anti-AQP4 antibody-positive MS.     

Double filtration plasmapheresis in the treatment of myasthenic crisis--analysis of prognostic factors and efficacy

OBJECTIVES: To examine the prognostic factors and outcome of myasthenia gravis (MG) patients in crisis with double filtration plasmapheresis (DFP) treatment. MATERIAL AND METHODS: A total of 15 patients experienced 20 episodes of crisis during the study period. Plasmapheresis was carried out using a double filtration METHOD: Demographic information, clinical features of crisis, and associated complications were analyzed. RESULTS: The median duration of crisis was 9 days. Chest infection was the most common precipitant of crisis. Twelve out of the 20 episodes (60%) responded well to DFP and mechanical ventilation was discontinued after the third session of DFP in 8 of them. Three significant predictors for prolonged crisis were shorter intervals between the onset of MG and the first crisis (P=0.04), higher serum bicarbonate levels at baseline (P=0.03) and the thymic pathology of thymoma (P=0.03). CONCLUSION: DFP can ameliorate the profound weakness in crisis and seems to be a rational therapy for patients with myasthenic crisis.     

Predicting the course of myasthenic weakness following double filtration plasmapheresis

OBJECTIVE: To evaluate the clinical course of patients with myasthenia gravis (MG) up to 3 months after double filtration plasmapheresis (DFP). MATERIAL AND METHODS: We recorded the MG score and measured the level of acetylcholine receptor antibody (AchRAb) at baseline and day 1 (D1), week 1 (W1), 1 month (M1), 2 (M2) and 3 months (M3) after DFP in 16 MG patients. Based on the difference in score during follow-up, we divided our patients into clinical improvement (CI) and clinical worsening (CW) groups. RESULTS: The MG score decreased in all courses from a mean of 8.1 at baseline to 5.6 at D1, and to 4.7, 4.0, 3.8, and 3.7 at W1, M1, M2, and M3, respectively. In the CW group, AchRAb levels were significantly higher at M1 (P = 0.022). The AchRAb level at W1 correlated significantly with the MG score at M3 (P = 0.027) and the changes of MG score from W1 to M1 (P = 0.029). The ratio of AchRAb levels of M1 to W1 correlated well with MG score at W1 (P = 0.032), at M3 (P = 0.001), and the changes of MG score from W1 to M1 (P = 0.004). CONCLUSION: Excessive rebounds of AchRAb level at W1 may suggest clinical worsening and further increases in AchRAb level at M1 predict poorer outcome after DFP.     

Double filtration plasmapheresis in myasthenia gravis - analysis of clinical efficacy and prognostic parameters

OBJECTIVES: The aim of this study was to evaluate the efficacy of double filtration plasmapheresis (DFP) in the treatment of patients with myasthenia gravis (MG) and to analyze the possible prognostic factors related to responsiveness to DFP. MATERIALS AND METHODS: We treated 45 MG patients, 26 women and 19 men aged 21-72 years, with DFP for 5 consecutive sessions. All were affected by severe generalized or respiratory weakness with an Osserman's classification of group 2 or 3 and had not responded to previous treatments. RESULTS: Thirty-eight out of 45 patients (84%) achieved significant improvements after DFP. The baseline MG score and removal rate for immunoglobulin G (IgG) were significantly higher in the patients with good response than in the other response groups. Poor responders were more likely to have thymoma and a longer interval among sessions of DFP. Better response in patients with age at onset of less than 40 years was associated with higher MG score. Serum concentration of all proteins tested fell as follows (mean +/- SD): IgM, 88+/-7%; IgA, 71+/-11%; IgG, 59+/-14%; globulin, 52+/-11%; AchRAb, 47+/-14%; and albumin, 27+/-10%. All the patients tolerated plasmapheresis well except for 2.2% who experienced hypotension. CONCLUSION: In this study, DFP was effective and safe in the treatment of patients with severe generalized MG. The factors correlating with the better clinical response were high MG score, a thymic pathology of non-thymoma, daily apheresis, young age at onset, and high removal rate for IgG.     

Comparison between double-filtration plasmapheresis and immunoadsorption plasmapheresis in the treatment of patients with myasthenia gravis

Two techniques for plasmapheresis are used in the treatment of myasthenia gravis (MG): immunoadsorption (IA) and double filtration (DR). This controlled study evaluated the differences between these techniques in clinical effects and serological changes. Five patients with generalized MG (clinical states IIb and III) were enrolled; each patient received IA and DF plasmapheresis on separate occasions. Immunosorba TR-350 with an affinity to acetylcholine receptor antibodies (AchRAb) was used for IA, while Evaflux 4A was used as the plasma fractionator for DF. Each course of treatment consisted of five sessions of apheresis. MG score, titers of AchRAb, immunoglobulins (IG), and plasma biochemistry were assessed by blinded examiners before and immediately after the entire course of treatment. Both treatments effectively ameliorated symptoms of MG. There were no significant changes in MG score between the two groups (IA vs. DF: 2.2 vs. 2.6, P>0.5). IA had a higher clearance rate of AchRAb than DF (66 % vs. 54 %, P<0.05), while DF removed more IgA (72 % vs. 21 %, P< 0.05) and IgM (89 % vs. 57 %, P<0.01) than did IA. Although IA removed AchRAb more effectively than DF, the clinical effects between these two treatments were similar. The titers of AchRAb cannot reflect the clinical severity. Some circulating factors other than AchRAb may contribute to the pathogenesis of MG. Received: 10 September 1999, Received in revised form: 7 February 2000, Accepted: 24 February 2000     

Changes in serum cytokine levels during plasmapheresis in patients with myasthenia gravis

Background: The effect of plasmapheresis on cytokine levels in patients with myasthenia gravis (MG) has not been well established. Methods: Cytokine levels were measured in 19 patients with MG before and after treatment with one course of double‐filtration plasmapheresis (DFP). The control group comprised 6 age‐ and sex‐matched healthy volunteers. Results: At baseline, patients with MG had higher levels of IL‐10 than normal controls. The levels of IL‐2, IL‐4, IL‐5, and tumor necrosis factor‐α were almost undetectable in MG patients. After a single session of DFP treatment, IL‐10 levels were significantly increased. After three sessions, IL‐10 levels were still higher than those at baseline. Elevated IL‐10 level was significantly associated with use of immunosuppressant drugs, thymectomy, and good response to DFP treatment. Conclusions: Interleukin‐10 might play a crucial role in the pathogenesis and perpetuation of MG.     

Th22细胞及相关因子在重症肌无力患者外周血中的表达

目的研究重症肌无力(myasthenia gravis,MG)患者外周血辅助性T细胞22(T helper 22cells,Th22)和白细胞介素-22(interleukin-22,IL-22)的表达以及两者间的相关性。方法收集25例MG患者和24例健康对照者,其中眼肌型重症肌无力(ocular myasthenia gravis,OMG)患者14例,全身型重症肌无力(general myasthenia gravis,GMG)患者11例。采用流式细胞仪检测MG患者和健康对照者外周血单个核细胞(peripheral blood mononuclear cells,PBMC)中Th22细胞的比例,采用酶联免疫吸附实验(enzyme-linked immunosorbent assay,ELISA)检测血浆IL-22的表达。比较各组间Th22细胞比例和IL-22表达水平差异,以及Th22细胞比例和IL-22表达间的相关性。结果 MG患者PBMC中Th22细胞比例、血浆IL-22表达水平均显著低于健康对照组[(0.60±0.07)%比(0.92±0.09)%,P0.01;(18.65±1.38)pg/mL比(24.54±1.85)pg/mL,P0.05];OMG与GMG患者间Th22细胞比例、IL-22表达水平均无统计学差异(均P0.05);MG患者PBMC中Th22细胞比例与IL-22表达水平间呈中度正相关(r=0.59,P0.01)。结论 MG患者体内Th22细胞比例及血浆IL-22表达水平减低可能导致免疫功能紊乱进而影响MG的发病。