重症肌无力患者血清IL-12水平变化及临床意义

目的观察重症肌无力(MG)患者血清白细胞介素12(IL-12)水平变化,并探讨其临床意义。方法采用双抗体夹心ELISA法检测69例MG患者(MG组)及30例健康体检者(NC组)血清IL-12、乙酰胆碱受体抗体(AchRab),AchRab>0.5 nmol/L为阳性;并对35例MG初治者采用糖皮质激素(GC)治疗3个月,同法检测治疗前后血清IL-12、AchRab。结果 MG组血清IL-I2、AchRab分别为(120.95±14.77)pg/mL、(0.69±0.10)nmol/L,NC组分别为(43.19±8.78)pg/mL、(0.37±0.09)nmol/L;两组比较,P均<0.01。AchRab阳性者血清IL-12为(123.07±16.72)pg/mL,高于AchRab阴性者的(110.90±14.72)pg/mL(P<0.01);MG患者血清IL-12水平与AchRab呈正相关(r=0.697,P<0.01)。伴胸腺影像学异常改变者血清IL-12、AchRab水平较均高于胸腺正常者(P均<0.01),但伴胸腺增生与胸腺瘤MG患者比较,P>0.05。GC治疗3个月后,35例MG初治者临床疗效绝对评分为(5.8±2.4)分,低于治疗前的(18.9±2.9)分(P<0.01);血清IL-12、AchRab水平亦均低于治疗前(P均<0.01),但仍均高于NC组(P均<0.01)。结论 MG患者血清IL-12水平升高,其有助于MG的病情及疗效判定。     

重症肌无力患者糖皮质激素受体的观察

目的 旨在观察重症肌无力（ＲＧ）患者外周血单个核细胞（ＰＢＭＣ）糖皮质激素受体（ＧＲ）的变化及其变化机制。方法 １９３例ＭＧ患者、１０全其他神经免疫性疾病患者、６例脑血管病患者及３５例正常对照;采用＾３Ｈ－氟美松放射配体法测定ＰＢＭＣ ＧＲ数目及亲和力,狭缝印迹法测定ＰＢＭＣ ＧＲｍＲＮＡ含量。结果 ＭＧ患者ＰＢＭＣ的ＧＲ数及其ｍＲＮＡ均显著降低（Ｐ〈０．０１）,而且ＧＲｍＲＮＡ的含量与ＧＲ数目密     

重症肌无力的免疫学和遗传学进展

重症肌无力(Myasthenia Gravis，MG)是乙酰胆碱能运动神经元与骨骼肌之间突触功能障碍性疾病，它是主要影响青壮年的一种自身免疫性疾病，近60％患者在20-40岁之间发病，50岁以后发病的情况少见。据估计，MG的年发病率约为每10万人口0．25—2．00人。     

重症肌无力患者糖皮质激素受体检测的临床价值

目的：探讨糖皮质激素受体（GR）数对预测糖皮质激素（GC）疗效的价值。方法：30例重症肌无力（MG）患者,采用^3H-地塞米松放射配体法测定外周血单个核细胞（PBMC）GR数目, 依据临床相对评分法评价GC治疗后1、6个月疗效,其中24例于GC治疗期间动态观察GR数的变化。结果：治疗前GR数与治疗后1、6个月临床相对评分间呈良好的正相关关系（r＝0．916和r＝0．891,P＜0．01）。治疗前GR数越高,临床相对评分越高,GC疗效越好。GC治疗后GR数降低。结论：用药前测定GR数可以作为预测GC治疗反应、确定治疗方案的一个指标。GC治疗可使GR数下调。     

重症肌无力患者外周血单个核细胞中糖皮质激素受体不同亚型表达和意义

目的探讨重症肌无力（MG）患者外周血单个核细胞（PBMC）中两种糖皮质激素受体（GR）亚型表达水平及其与糖皮质激素疗效的关系。方法在糖皮质激素治疗之前留取MG患者PBMC并涂片,根据糖皮质激素治疗MG的不同疗效分组,通过免疫细胞化学方法观察各组GRα和GRβ阳性细胞比例并评分,分析GRα和GRβ表达量与糖皮质激素疗效的关系。结果对照组、糖皮质激素治疗敏感组和依赖组PBMC中GRα阳性细胞计分差异无统计学意义,3组计分明显高于糖皮质激素治疗抵抗组（P〈0．01）;对照组、糖皮质激素治疗敏感组和糖皮质激素治疗抵抗组PBMC中GRβ计分差异无统计学意义,3组计分明显低于糖皮质激素治疗依赖组（P〈0．01）。结论MG患者PBMC中GRα表达降低和GRβ表达增高均与糖皮质激素疗效降低有关。     

糖皮质激素治疗对重症肌无力患者BAFF和IL-6水平的影响

目的 探讨糖皮质激素(GC)对新发病重症肌无力(MG)患者外周血B淋巴细胞刺激因子(BAFF)、白介素6(IL-6)水平及临床疗效的影响.方法 采用ELISA法检测26例MG患者GC治疗前、后及正常对照组血清BAFF、IL-6水平,并观察GC治疗的临床效果.结果 MG患者外周血清IL-6、BAFF水平明显高于正常对照组水平(P<0.05),激素治疗2个月后,MG患者外周血BAFF、IL-6水平显著下降(t=9.79 or t=3.45,P<0.01),治疗前后IL-6变化与肌无力相对评分存在显著正相关性(r=0.559,P<0.01).结论 MG患者血清IL-6水平可能与病情严重程度呈正相关,GC可通过抑制IL-6、BAFF分泌,有效调节MG患者细胞免疫功能.     

重症肌无力的治疗进展

重症肌无力(myasthenia gravis,MG)是乙酰胆碱受体抗体(AchR-Ab)介导的,细胞免疫依赖及补体参与的神经-肌肉接头(NMJ)处传递障碍的自身免疫性疾病.临床特征为受累骨骼肌易疲劳,呈波动性肌无力,具有活动后加重、休息后减轻和晨轻暮重等特点.该病慢性迁延、反复发作,严重时导致呼吸肌麻痹,危及生命.但只要得到及时、合理的治疗,绝大多数患者症状可以减轻甚至完全缓解.现今治疗方式主要是针对抗体、淋巴细胞和胸腺组织的免疫干预治疗以及对症治疗.本文对近年来采用的治疗进展做一综述.     

免疫吸附疗法治疗重症肌无力的进展

重症肌无力(myasthenia gravis,MG)是由乙酰胆碱受体抗体(acetylcholine receptor antibody,AchRab)介导、细胞免疫依赖及补体参与的神经肌肉接头处传递障碍的自身免疫性疾病.自从认识到AchRab在MG致病机制中起关键作用后,以直接清除MG患者血中的致病抗体为目的的血液净化治疗受到重视并得到很快的发展.     

胸腺切除术对重症肌无力患者AchRb及PsmRAb的影响

用标准经胸胸腺切除术治疗重症肌无力（MG）患者,术前及术后3个月,采用酶联免疫吸附法检测其血清乙酰胆碱受体抗体（AchRAb）、抗突触前膜受体抗体（PsmRAb）滴度的变化。结果MG胸腺增生患者术后Ach-RAb滴度低于术前（P〈0．01）,手术前后PsmRAb无统计学差异;MG胸腺瘤患者,手术前后AchRAb无统计学差异,但术后PsmRAb高于术前（P〈0．01）。提示胸腺切除后,MG胸腺增生患者AchRAb下降,MG胸腺瘤患者PsmRAb升高。     

重症肌无力危象20例临床分析

目的 总结重症肌无力危象的临床特点及急救和预防经验.方法 回顾性分析1998年-2008 年我院神经内科诊治的82例重症肌无力患者20例重症肌无力危象的病例资料.结果 死亡1人,病死率1.22%.综合利用气管切开正压辅助呼吸、激素治疗、丙种球蛋白静脉输注、胆碱酯酶抑制剂、抗生素预防感染等可改善重症肌无力危象的预后,且明显降低病死率.结论 发生危象的主要为Osserman Ⅲ型和Ⅳ型患者.感染发热性疾病及加、减药量过快或突然停药为危象发生的主要诱因.适当调整胆碱酯酶抑制剂的用量,联合应用甲泼尼龙和丙种球蛋白冲击,注意控制感染等综合措施治疗,可使肌无力危象病死率明显降低.     

重症肌无力患者妊娠期临床特点及预后分析

目的:探讨重症肌无力（MG）患者妊娠前后肌无力症状的变化及MG对妊娠预后的影响。方法:回顾性研究2013年1月至2018年10月28例就诊于解放军总医院第八医学中心MG患者38例次妊娠分娩的临床资料。比较妊娠前与妊娠不同时期MG严重程度评分、血乙酰胆碱受体（AChR）抗体变化及药物的使用情况,并分析妊娠后MG不同转归（...     

232例老年人重症肌无力的临床分析

目的:分析老年人重症肌无力(MG)的有关临床特点。方法:回顾性分析26年间诊治的232例老年MG患者的临床资料。结果:老年人MG占总MG病例数(3010例)的比例为7．7％。男性多于女性，男：女=1．3：1。老年人MG多在60～70岁间发病，在其常见首发症状中，眼症状184例(79．3％)、咽喉肌无力30例(12．9％)、肢体肌肉无力17例(7．3％)。老年人MG中，全身型的构成比多于眼型(62．9％对37．1％)，较少并存自身免疫性疾病和发生危象，常合并胸腺瘤(33例)及内外科其他疾病(87例)。结论:老年人MG具有独特的临床特点，了解这些特点将有利于指导临床诊断和治疗。     

Adsorption column for myasthenia gravis treatment: Medisorba MG-50

Adsorption column Medisorba MG-50 (Kuraray Medical Inc.) for the treatment of myasthenia gravis (MG) is introduced. The adsorbent in this column is composed of cellulose beads as carrier material and covalent-bound synthetic peptide as a ligand that has a specific affinity to the pathogenic anti-acetylcholine receptor antibody of MG. The amino acid sequence of the peptide is modified from the segment of alpha 183-200 of the torpedo acetylcholine receptor (AChR) protein, and the segment is the acetylcholine binding site on AChR and the target site of anti-AChR antibody. The adsorbent showed specific adsorption characteristics to the anti-ACHR antibody (blocking antibody) in vitro. Clinically, MG-50 is used in plasma-perfusion therapy, and it is recognized that MG-50 specifically reduces blocking antibody titer and improves MG symptoms. MG-50 is approved in Japan.     

Adult celiac disease with acetylcholine receptor antibody positive myasthenia gravis

Celiac disease has been associated with some autoimmune disorders. A 40-year-old competitive strongman with celiac disease responded to a glutenfree 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.     

The co-existence of myasthenia gravis in patients with myositis: A case series

ObjectiveMyositis and myasthenia gravis (MG) are both autoimmune disorders presenting with muscle weakness. Rarely, they occur simultaneously in the same patient. Since the management of myasthenia gravis differs from that of myositis, it is important to recognize when patients have both diseases. We reviewed the cases of 6 patients with both myositis and MG to identify clinical features that suggest the possibility of co-existing MG in myositis patients.MethodsWe identified 6 patients with dermatomyositis or polymyositis and MG. We reviewed their medical records to assess their clinical presentations, laboratory findings, and electrophysiological features.ResultsAll 6 patients had definite dermatomyositis or polymyositis by the criteria of Bohan and Peter as well as electrophysiologic and/or serologic confirmation of MG. Among overlap patients, 5/6 (83%) had bulbar weakness, 2/6 (33%) had ptosis, and 1/6 (17%) had diplopia. Fatigable weakness was noted by 5/6 (83%) patients. Treatment with pyridostigmine improved symptoms in 5/6 (83%) patients. High-dose steroids were associated with worsening weakness in 2/6 (33%) patients.ConclusionsProminent bulbar symptoms, ptosis, diplopia, and fatigable weakness should suggest the possibility of MG in patients with myositis. A suspicion of MG may be confirmed through appropriate electrophysiologic and laboratory testing. In those with myositis–MG overlap, high-dose steroids may exacerbate symptoms and pyridostigmine may play an important therapeutic role.     

氨溴索对老年重症肌无力患者围术期调控炎症反应及肺保护的作用研究

目的 探讨氨溴索在老年重症肌无力患者围术期的肺保护作用. 方法 收集58例老年重症肌无力并胸腺疾病患者按入组编号随机分为观察组与对照组,每组29例,治疗组在围术期应用盐酸氨溴索干预,而对照组不予盐酸氨溴索干预,观察两组患者术后肺部并发症、肺通气指标、血气分析,并检测血清C反应蛋白(CRP)、肿瘤坏死因子α(TNF-α)、白介素(IL)1β和IL-10的浓度.结果 (1)治疗组患者肺部并发症(肺不张、肺炎和支气管炎)发生率低于对照组(9.4％和15.6％,P＜0.05);(2)治疗组肺通气指标气管峰压[(22.32±3.43) cmH2O和(26.54±4.81) cmH2O,P＜0.05]和气道阻力[(29.17±5.74)cmH2O·L-1·S-1和(34.47±7.94)cmH2O·L-1·S-1,P＜0.05]较对照组降低,肺顺应性较对照组升高[(106.04±14.73) ml/cmH2O和(95.11±9.50) ml/cmH2O,P＜0.05];(3)术后第2天,治疗组的动脉氧分压[(89.66±13.23) mmHg和(70.94±12.75)mmHg,P＜0.05]、血氧饱和度[(96.95±2.65)％和(89.44±2.78)％,P＜0.05]和氧合指数[(219.47±54.05)％和(187.38±37.72)％,P＜0.05]均高于对照组;(4)术后3d治疗组血清CRP[(29.37±14.87)mg/L和(43.94±21.42) mg/L,P＜0.05]、TNF-α[(35.55±4.74) μg/L和(52.80±6.63) μg/L,P＜0.05]及IL-1β[(17.06±6.85)μg/L和(28.62±7.72) μg/L,P＜0.05]均低于对照组,而血清IL-10[(132.84±12.91)μg/L和(87.18±16.37) μg/L,P＜0.05]高于对照组. 结论 盐酸氨溴索可减少老年重症肌无力患者胸腺切除术后呼吸道并发症的发生率,改善肺通气功能,有效抑制炎症反应,值得在老年重症肌无力患者围术期中推广应用.     

The co-existence of myasthenia gravis in patients with myositis: A case series

Objective

Myositis and myasthenia gravis (MG) are both autoimmune disorders presenting with muscle weakness. Rarely, they occur simultaneously in the same patient. Since the management of myasthenia gravis differs from that of myositis, it is important to recognize when patients have both diseases. We reviewed the cases of 6 patients with both myositis and MG to identify clinical features that suggest the possibility of co-existing MG in myositis patients.

Methods

We identified 6 patients with dermatomyositis or polymyositis and MG. We reviewed their medical records to assess their clinical presentations, laboratory findings, and electrophysiological features.

Results

All 6 patients had definite dermatomyositis or polymyositis by the criteria of Bohan and Peter as well as electrophysiologic and/or serologic confirmation of MG. Among overlap patients, 5/6 (83%) had bulbar weakness, 2/6 (33%) had ptosis, and 1/6 (17%) had diplopia. Fatigable weakness was noted by 5/6 (83%) patients. Treatment with pyridostigmine improved symptoms in 5/6 (83%) patients. High-dose steroids were associated with worsening weakness in 2/6 (33%) patients.

Conclusions

Prominent bulbar symptoms, ptosis, diplopia, and fatigable weakness should suggest the possibility of MG in patients with myositis. A suspicion of MG may be confirmed through appropriate electrophysiologic and laboratory testing. In those with myositis–MG overlap, high-dose steroids may exacerbate symptoms and pyridostigmine may play an important therapeutic role.     

免疫治疗对重症肌无力病人淋巴细胞亚型的影响

目的 研究重症肌无力（ＭＧ）患者细胞免疫的异常改变和糖皮质激素及胸腺切除对各淋巴细胞亚群的影响。方法 采用免疫荧光双标记技术和流式细胞仪对３９例ＭＧ患者和１８例健康对照者周围血中淋巴细胞表型分析,并对１５例ＭＧ患者经患者经糖皮质激素治疗前后和７例ＭＧ患者在胸腺切除前后淋巴细胞亚群的百分率的变化进行观察。结果（１）ＭＧ患者总Ｔ细胞,总Ｂ细胞（ＣＤ１９＾＋）,传统Ｂ细胞（ＣＤ５＾－ＣＤ１９＾＋）,ＣＤ     

他克莫司治疗重症肌无力疗效及安全性评价

目的 评价他克莫司治疗全身型重症肌无力(MG)的疗效及不良反应.方法 回顾性分析69例全身型MG患者接受他克莫司(2～6 mg/d)治疗前及治疗后1、3、6及12个月时MG严重程度评分及不良反应,并监测服药1个月后他克莫司血药浓度(FK506),分析其与临床疗效的相关性.结果 他克莫司治疗1、3、6及12个月的总有效率分别为81.2％、87.6％、92.2％及93.8％.治疗1个月后,临床显效及好转组FK506[(7.1±3.9) μg/L,(6.3±3.8) μg/L]明显高于无效组[(3.4±1.3) μg/L] (P ＜0.01,P＜0.05).主要的不良反应:血糖升高5例,出现白细胞减少及头晕、耳鸣各3例.结论 他克莫司治疗MG,起效快,临床疗效确切.在治疗剂量范围内其副作用小,主要为血糖升高及骨髓抑制.     

重症肌无力伴骨骼肌以外受损的临床特征分析

目的 对伴心脏、肝脏损害,瞳孔不等,听力障碍,手足血管舒缩功能及皮肤营养障碍和阳痿等少见症状的重症肌无力(MG)患者的特点进行分析,提出诊断和鉴别诊断的方法。方法 (1)给予MG患者胆碱酯酶抑制剂(ChEI)及免疫治疗,再复查病情和随访;(2)伴瞳孔不等和听力障碍患者肌注新斯的明1mg,30～120min后观察症状变化,后者同时检查脑干听觉诱发电位2次。结果 55例MG患者临床症状随典型的骨骼肌病态疲劳的症状改善而好转,一般不需要特殊治疗。结论 ChEI治疗MG患者合并上述少见症状均有效,是与其他疾病鉴别的重要依据,也证实了MG是一种广泛的自身免疫性疾病。