老年起病型重症肌无力患者的临床特点

目的探讨老年起病型重症肌无力(MG)的临床特点。方法回顾性分析332例起病年龄≥65岁的MG患者的临床资料。结果该组MG患者男女比例为1.3∶1。全身型MG (GMG)和眼肌型MG(OMG)的比例为1.9∶1,OMG在男性多于女性而GMG在女性多于男性(P=0.033)。以OMG起病和以GMG起病的比例为1.5∶1,起病症状以眼睑下垂最多见占38.6%;在以OMG起病的患者中,女性患者转化为GMG的比率要明显高于男性患者(P=0.035)。病程中位数为3.7年,平均定量MG评分为7分。新斯的明试验阳性率为96.7%。低频重复频率电刺激波幅递减的总体发生率为48.2%,其中面神经的阳性率最高。MG合并胸腺异常的发生率为36.3%,以合并胸腺瘤者最多;胸腺瘤手术后病理分型以AB型最多,女性患者AB型胸腺瘤所占的比率要明显高于男性(P=0.048)。MG合并甲状腺功能异常的发生率为16%,以合并甲状腺功能亢进者最多。结论老年起病型MG患者GMG多于OMG、以OMG起病的女性患者转化为GMG的比率高于男性患者,胸腺瘤是最常见的胸腺异常且最常见的胸腺瘤病理分型为AB型。充分认识其临床特点有利于更好的治疗该类患者。     

吉兰-巴雷综合征30例临床分析

目的分析吉兰-巴雷综合征的临床特点。方法回顾性分析我院收治的30例吉兰-巴雷综合征住院患者的临床资料、实验室检验结果、治疗及预后。结果吉兰-巴雷综合征患者男性多发,且随年龄增长发病率升高;起病前有呼吸道或消化道感染史9例,脑脊液实验室检查正常7例,症状不典型误诊3例,肌电图检查阳性率100%;丙种球蛋白治疗有效率80%(20/25);肢体无力严重、合并呼吸肌麻痹以及治疗延后的患者疗效不佳占50%(7/14)。结论不典型症状起病的吉兰-巴雷患者易误诊,丙球治疗疗效好但需及时,肌无力重、合并呼吸肌麻痹患者预后差。     

早、晚发型重症肌无力患者临床特点对比研究

目的研究早、晚发型重症肌无力(MG)患者的临床特点。方法回顾性研究同期住院及门诊的191例MG患者资料,比较早、晚发型MG组患者的构成情况、首发症状、临床分型、伴随疾病、辅助检查、治疗及疗效等临床特点。结果 MG发病以晚发型人群为主,占63.35%。早发型MG组中,男∶女=1∶1.80;晚发型组中,男:女=1:0.92。两组患者首发症状及osserman分型构成比无统计学差异(P0.05),都以Ⅱ型居多,约占早发型58.57%,晚发型57.85%,但晚发型组Ⅱb型较早发型组比例高。晚发型伴随高血压、2型糖尿病、高脂血症比例高(分别42.98%和8.57%,23.97%和2.86%,13.22%和0%;P0.05),而胸腺瘤、甲状腺疾病、其他免疫疾病、肿瘤及副肿瘤综合征在两组患者中差异无统计学意义(P0.05)。早发型MG患者伴胸腺增生构成比例高,晚发型伴胸腺瘤构成比例高,男性患者胸腺瘤占比例高,女性患者胸腺增生占比例高,差异均有统计学意义(P0.05)。女性MG患者伴随甲状腺疾病比例高(P0.05)。两组患者行血清Titin-Ab检查,早发型患者阳性率较晚发型高(P0.05),余血清抗体检测、电生理检查和新斯的明试验比较,差异均无统计学意义(P0.05)。两组患者治疗有效率差异无统计学意义(P0.05),但晚发型有效率较早发型低。结论早、晚发型MG患者在性别构成、首发症状、伴随疾病、辅助检查及预后有所不同,在诊断及治疗时需要注意。     

Bickerstaff脑干脑炎和Miller Fisher综合征临床及预后的对比研究

目的探讨Bickerstaff脑干脑炎(BBE)和Miller Fisher综合征(MFS)的临床特征,研究两者的症状学特点及疾病分类学关系。方法对湘雅二医院2003-01-2013-12符合BBE(21例)和MFS(67例)诊断标准的患者的临床资料进行回顾性分析。结果 BBE和MFS患者均存在以上呼吸道感染为主的前驱感染症状〔10例(48%)、36例(54%)〕,并具有眼外肌麻痹和共济失调的共同症状〔21例(100%)、67例(100%)〕,头痛〔8例(42%)、20例(30%)〕、眼内肌麻痹〔8例(38%)、17例(25%)〕、延髓麻痹〔8例(50%)、32例(48%)〕、面瘫〔6例(35%)、20例(30%)〕、浅表感觉障碍〔4例(25%)、28例(42%)〕等症状在两者中均常见且发生率相近;两者均有脑脊液蛋白-细胞分离现象〔5例(25%)、23例(38%)〕和头部影像学检查〔10例(59%)、2例(4%)〕异常。两者主要的不同点在于BBE患者的中枢神经系统受累表现如意识障碍〔16例(76%)〕、腱反射亢进〔11例(52%)〕、Babinski征〔6例(32%)〕、头部MRI脑干部位异常信号〔10例(59%)〕、脑电图异常〔7例(78%)〕较MFS患者〔0例、0例、8例(12%)、2例(4%)、0例〕常见。两组患者预后均较好,随访期间(≥1年)均未见复发病例,MFS患者59例(88%)症状完全消失,BBE患者10例(48%)症状完全消失,3例(14%)明显改善,6例BBE死亡。结论BBE与MFS临床特点相似且存在交叉重叠,两者可能形成同一连续性自身免疫性疾病谱。     

重症肌无力胸腺基质淋巴细胞生成素表达与CD4~+CD25~+Foxp3~+Treg细胞表型的研究

目的探索重症肌无力患者胸腺基质淋巴细胞生成素(TSLP)表达水平与CD4+CD25+Foxp3+调节性T细胞(Treg)表型的相关性。方法 MG组(16例经胸腺切除的MG患者)及对照组(23例先天性心脏病心脏手术后患者)取外周血单个核细胞,经CD4+CD25+抗体表面染色后加入破膜剂孵育,以Foxp3+抗体行胞内染色,以流式细胞技术检测CD4+CD25+Foxp3+Treg/CD4+T细胞比率;同时取两组患者对应的切除胸腺组织,以免疫组织化学法检测TSLP表达水平,并进行两组间比较;以Logistic回归分析方法分析TSLP阳性表达的Hassall小体计数与Treg细胞之间的相关性。结果CD4+CD25+/CD4+T细胞比率MG组〔(6.24±0.62)%〕与对照组〔(6.56±0.65)%〕无统计学差异(P>0.05),MG组CD4+CD25+Foxp3+Treg/CD4+T细胞比率〔(3.82±0.49)%〕较对照组〔(5.73±0.56)%〕明显降低(P<0.01);与对照组比较,MG组患者胸腺TSLP阳性面积大,染色深,且TSLP阳性的Hassall小体数目(6.81±2.17)明显低于对照组(18.87±3.06)(P<0.01)。MG组TSLP阳性表达的Hassall小体计数与Treg细胞表达量之间呈线性相关(R2=0.158,F=13.42,P<0.01)。结论 MG患者TSLP表达不足与胸腺Treg细胞发育过程中CD4+CD25+Foxp3+表型的表达缺陷呈正相关。     

重症肌无力合并自身免疫病的临床研究

目的分析重症肌无力(MG)合并自身免疫病的种类、发病年龄和临床特点。方法回顾性分析作者医院2008-01-2012-01住院的MG患者,包括合并自身免疫病的MG患者的临床资料、影像学及实验室检查资料及治疗记录。结果共收集MG患者305例,合并自身免疫病1种或1种以上者28例(9.18%),其中男9例、女19例,性别构成与非合并免疫病者无统计学差异(P>0.05)。自身免疫病在MG之前发病者12例,平均(69.6±35.3)个月;在MG发病之后者14例,平均(72.4±38.4)个月;与MG同时发现2例;(1)MG合并自身免疫病组的MG发病年龄为(33.9±9.4)岁,与非合并自身免疫病患者的MG发病年龄〔(35.3±12.2)岁〕无统计学差异(P>0.05)。合并甲状腺功能亢进(甲亢)患者MG发病年龄(27.4±12.5)岁,较合并其他自身免疫病患者的MG发病年龄〔(41.6±10.3)岁〕和非合并自身免疫病患者(35.3±12.2岁)均低(均P<0.05)。(2)合并的自身免疫病中属甲亢最多(13例),其次为类风湿性关节炎(5例)、桥本甲状腺炎(4例)、吉兰-巴雷综合征(3例)。其中有3例合并2种自身免疫病,1例合并3种自身免疫病。(3)合并自身免疫病MG患者的Osserman分型:发病初期Ⅰ型13例,Ⅱa型3例,Ⅱb型10例,Ⅲ型2例;其中MG合并甲亢患者以Ⅰ型发病10例,超过合并其他自身免疫病MG患者(P<0.05)。结论 MG可合并多种神经系统或全身自身免疫病,以甲亢最多见。合并甲亢的MG患者中MG发病年龄较轻,以眼肌型多见。自身免疫病可在MG发病前、后或同时期出现。     

老年重症肌无力住院患者48例临床特点分析

目的分析老年重症肌无力(MG)住院患者的有关临床特点。方法回顾性分析近年来在我院住院诊治的48例老年MG患者的临床资料。结果老年MG患者占总住院MG病例数(175例)的比例为27.4%。男性多于女性,男∶女=2.7∶1。老年MG多在60~69岁之间发病,在其常见首发症状中,眼部症状最多,为34例(70.8%)、球麻痹其次,为8例(16.7%)、肢体肌无力5例(10.4%)、肌萎缩1例(2.1%)。老年MG患者中,全身型比例(70.8%)显著高于眼肌型(29.2%),较少合并自身免疫性疾病和发生危象,常合并胸腺病变(17例)及其他内外科疾病(36例),其中15例为胸腺瘤。伴胸腺病变的老年MG患者出现首发症状的年龄与无胸腺病变的老年MG患者比较差异无统计学意义;而伴胸腺病变的老年MG患者需住院治疗时的年龄显著小于无胸腺病变的老年MG患者,差异有统计学意义(P0.05)。结论老年MG具有其自身特点,男性多见,早期表现多为眼肌无力,全身型比例高于眼肌型,胸腺病变主要为胸腺瘤,危象发生率较低等临床特点。     

急性脑梗死患者的睡眠障碍研究

目的观察急性脑梗死患者睡眠障碍特点。方法选择首次发作的急性脑梗死患者101例(脑梗死组),以本院非脑梗死患者86例为对照,采用匹兹堡睡眠质量指数(PSQI)及爱泼沃斯嗜睡量表(ESS)进行睡眠量表评估,并行多导睡眠监测,观察总睡眠时间(TST)、睡眠效率(SE),第一、二、三及四期睡眠(S1、S2、S3及S4,S3+S4为深睡眠时间)、快速眼动睡眠时间(REM)、觉醒期时间(WASO)、睡眠潜伏期时间(SL)及快动眼睡眠潜伏期(RL)等情况。S1、S2、S3+S4、REM及WASO分别记录为占总睡眠时间的百分数。分析脑梗死患者睡眠障碍的发病率及特点,并比较脑梗死患者中,不同性别患者,不同大脑半球梗死患者,前、后循环梗死患者,以及丘脑和其他非丘脑部位梗死患者睡眠障碍情况。结果 (1)脑梗死组睡眠障碍的发生率〔77.23%(78/101)〕高于对照组〔27.91%(24/86);t=6.31,P=0.00〕。脑梗死组睡眠呼吸紊乱比例〔65.35%(66/101)〕高于对照组〔11.62%(10/86);t=36.50,P=0.00〕。(2)脑梗死组患者TST为(31.07±91.33)min,S1为(14.70±14.59)%,S3+S4为(10.30±7.30)%,PSQI为(9.10±4.80),均少于对照组〔分别为TST(421.00±100.70)min、S1(24.07±19.12)%、S3+S4(11.40±6.70)%、PSQI(14.70±5.90),均P0.05〕;脑梗死组WASO为(19.55±22.96)%,ESS为(6.30±5.20),REM为(59.51±49.31)%,SL(46.80±34.60)min,RL为(136.00±87.30)min,均多于对照组〔分别为WASO(6.92±14.05)%、ESS(1.00±1.60)、REM(38.39±52.02)%,SL(31.30±30.60)min,RL(114.00±75.30)min,均P0.05〕。(3)女性患者TST为(262.50±87.00)min,REM为(11.90±12.07)%,SL为(11.00±5.30)min,均少于男性〔分别为TST(328.00±104.80)min、REM(13.60±10.06)%、SL(35.00±11.20)min,均P0.05〕;女性患者S1为(15.70±13.70)%,RL(83.00±12.60)min,均多于男性〔分别为S1(7.90±9.30)%和RL(22.00±7.90)%,均P0.01〕。(4)左侧半球梗死者S1为(16.80±16.44)%,ESS为(7.41±6.50),均多于右侧半球梗死者〔分别为S1(10.29±9.39)%、ESS(4.85±4.09),均P0.05〕。(5)前循环梗死组患者S3+S4、REM〔分别为(17.36±14.57)%、(9.22±1.68)%〕亦均较后循环梗死组〔分别为(32.11±17.89)%、(16.36±13.66)%〕减少(均P0.05)。(6)丘脑组S2〔(32.56±18.70)%〕、SL〔(10.30±4.98)min〕均低于非丘脑梗死组〔分别为(40.39±13.94)%和(23.76±10.73)min〕,丘脑组S3+S4〔(46.03±27.01)%〕高于非丘脑组〔(41.25±17.61)%〕(均P0.05)。结论急性脑梗死患者睡眠障碍发生率高,可出现多种类型的睡眠障碍及睡眠结构紊乱,且不同性别、不同梗死部位患者睡眠障碍情况存在差异。     

以眼肌麻痹为唯一或主要表现的脑干梗死临床特点研究

目的研究以眼肌麻痹为唯一或主要表现的脑干梗死患者临床和影像学表现、预后及探索其病因及发病机制。方法回顾性连续收集2010年-2016年间在我院神经内科经临床和影像学诊断证实的20例以眼肌麻痹为唯一或主要表现的脑干梗死住院患者的临床资料,分析眼肌麻痹的类型、伴随神经系统症状和体征、病灶特点、责任血管、危险因素及疾病预后。结果 20例以眼肌麻痹为唯一或主要表现的脑干梗死患者的眼肌麻痹表现为:核下性(1例),核性(9例),核间性(10例)。核性眼肌麻痹以动眼神经不全麻痹最常见(78%);核间性眼肌麻痹以前核间性眼肌麻痹多见(60%)。孤立性眼肌麻痹患者6例,伴随症状以头晕最多见(55%)。18例(90%)患者伴颅内多发腔隙性病灶,磁共振血管成像(magnetic resonance angiography,MRA)显示与梗死灶相关的血管狭窄或闭塞有14例(70%),常见为大脑后动脉狭窄12例(60%)。13例(65%)在住院期间眼部症状消失,消失时间以发病后2 w内最多见(50%)。经随访,14例(70%)患者在发病后3 m内眼部症状消失。比较孤立性眼肌麻痹组(n=6)与非孤立眼肌麻痹组(n=14)临床、影像特点及实验室指标方面差异,非孤立眼肌麻痹组空腹血糖值更高,差异有统计学意义(P0.05)。结论脑干梗死引起的眼肌麻痹可有各种表现,以核间性眼肌麻痹最常见,核性眼肌麻痹以动眼神经不全麻痹多见。多数患者伴后循环大血管狭窄或闭塞。大部分患者预后良好,眼部症状多在发病后3 m内消失。高血糖可能导致更广泛的神经功能缺损。     

肾病综合征合并颅内静脉系统血栓形成临床特点及其可能机制

目的探讨肾病综合征(primary nephritic syndrome,PNS)合并颅内静脉系统血栓形成(erebral venous sinus thrombosis,CVST)的临床特点及可能的发病机制。方法回顾性调查分析2006年1月~2015年12月在广西北海市人民医院住院治疗的PNS合并CVST患者的临床资料。结果符合PNS合并CVST条件的患者共26例,占同期962例PNS患者的2.70%,26例患者中男12例(46.15%),女14例(53.85%),年龄在10~52岁,平均(33.25±3.8)岁。本组患者均缺少CVST常见的危险因素,4例(15.38%)以CVST为首发,其余均在PNS诊断后1y内发病。所有患者均呈急性、亚急性起病,临床表现以头痛、运动和(或)感觉障碍、癫痫、不同程度意识障碍为常见;血液生化检查显示低蛋白血症、血小板计数增多、D-二聚体水平升高常见;受累静脉窦以上矢状窦最常见(12例,46.15%)。患者CVST起病后30 d预后多数良好,少数出现中度以上功能障碍(mRS=3~5)2例(7.69%),1例死亡(3.85%)。结论 PNS合并CVST以缺少常见的危险因素,血液生化异常、D-二聚体水平升高、颅内上矢状窦受累多见等为临床特点;其发病机制可能与患者的血液处于高疑状态有关。     

应用视频眼震电图分析新斯的明试验中的睑裂变化

目的 应用视频眼震电图定量分析新斯的明试验中的睑裂变化.方法 对35例重症肌无力(MG)患者、21例非MG(其他疾病)患者以及23名健康对照者进行了新斯的明试验,并利用视频眼震电图检查系统测量试验前及试验后1 h(每10分钟1次)内受试者睑裂大小的变化.使用重复测量方差分析同一组内不同时间点之间及不同组间的睑裂差异.结果 MG组组内不同时间点睑裂大小变化(mm)差异有统计学意义(试验前:4.67±1.87;试验后10 min:0.88±0.96,20 min:1.49±1.38,30 min:1.71±1.53,40 min:1.77±1.82,50 min:1.79±1.52,60 min:1.62±1.68;F=11.202,P=0.002);MG组与非MG组及MG组与健康对照组间睑裂变化比较差异均有统计学意义(F=15.569,P＜0.01;F=15.104,P＜0.01).各组变化率的改变及统计学意义也具有同样的特点.受试者工作曲线分析表明,1.17 mm(变化值)或17.5％(变化率)对新斯的明试验有较好的诊断能力.结论 视频眼震电图检查系统为新斯的明试验中睑裂大小的观察提供了客观、可靠的测量方法.睑裂变化值大于1.17mm或变化率大于17.5％者高度怀疑MG的可能.     

Myasthenia gravis Lambert‐Eaton overlap syndrome

Introduction: To assess whether a myasthenia gravis (MG) Lambert‐Eaton overlap syndrome (MLOS) exists. Methods: Case reports that met the universally accepted diagnostic criteria for MG and Lambert‐Eaton myasthenic syndrome (LEMS) were sought through a PubMed search. Fifty‐five possible cases of MLOS were identified. Results: Thirty‐nine cases met the diagnostic criteria for MG and LEMS. Analysis of clinical features showed that these patients have common MG and LEMS symptoms: oculo‐bulbar paresis and good response to anti‐cholinesterase for MG and limb weakness and decreased or absent reflexes for LEMS. All had the classical LEMS pattern in the repetitive nerve stimulation test: low compound muscle action potential amplitude and incremental response > 60% with brief exercise or at high rate of stimulation. Eight patients had combined positive acetylcholine receptor antibody (AChR‐ab) or muscle‐specific kinase‐ab and voltage‐gated calcium channel‐ ab tests. Conclusions: A myasthenia gravis Lambert‐Eaton overlap syndrome (MLOS) does exist. Muscle Nerve 53 : 20–26, 2016     

重症肌无力患者针电极肌电图肌源性受损表现的临床意义

目的分析重症肌无力(myasthenia gravis,MG)患者针电极肌电图(needle electrode electromyography,NEMG)检查结果的临床意义。方法回顾性分析2011-01-01—2013-12-31期间在解放军第309医院神经内科住院治疗的335例确诊MG患者的NEMG检查结果和临床资料,根据NEMG检查是否出现肌源性受损表现将患者分为两组,对比分析两组患者的临床特点。结果29例(8.7%)NEMG出现肌源性受损表现,异常NEMG均无自发电位,仅表现为运动单位电位(motor unit potential,MUP)波幅降低、时限缩短。NEMG检查有肌源性受损表现组临床绝对评分(20.8±7.3)高于无肌源性受损表现组(14.9±9.0,t=1.79,P0.05)。NEMG检查无肌源性受损表现者多以眼外肌无力为首发症状(85.62%),以肢体和球部肌肉起病者比例较低(14.38%);与无肌源性受损表现者相比,有肌源性受损表现者以眼外肌为首发症状者比例较低(55.17%),多以肢体和球部肌肉受累起病(44.83%),两组差异有统计学意义(χ2=9.79,P0.01)。两组间比较,性别、发病年龄、病程、Osserman分型及胸腺病理类型差异均无统计学意义(均P0.05)。结论 NEMG检测表现为肌源性受损者病情较无肌源性受损表现者重。电生理检查可在一定程度上提示MG病情的严重程度。     

Single-fiber electromyography of masseter muscle in myasthenia gravis

Jitter after axonal microstimulation in the masseter muscle was studied in 30 consecutive patients (12 women) with myasthenia gravis (MG). Patients' mean age was 42.3 (12-75), median disease duration was 3 months (1-72), and onset was ocular (15 cases), oculobulbar (7), bulbar (6), or generalized (2). There were 23 newly-diagnosed patients. Nine cases developed purely ocular MG and 21 cases developed generalized MG. In the latter group, five subjects had a rapidly progressive course and 16 subjects had stable or well-controlled disease (MGFA grade 2-3). Six patients did not have circulating anti-acetylcholine receptor antibodies. Masseter single-fiber electromyography (SFEMG) was abnormal in 6 of 9 ocular MG patients and in all generalized cases (overall sensitivity 27 of 30 cases or 90%; confidence interval 79.3%-100.0% at P = 0.95). Masseter should be considered for SFEMG in diagnosis of MG, especially in cases with bulbar onset.     

Late onset generalized myasthenia gravis presenting with facial weakness and bulbar signs without extraocular muscle involvement

Bilateral facial palsy is a common sign of autoimmune myasthenia gravis (MG). However, facial muscle weakness without clinical signs of extraocular muscle (EOM) involvement is extremely rare. We describe a late onset generalized MG presenting with facial weakness and bulbar signs without EOM deficits. The diagnosis was based on neurophysiological assessment and positive acetylcholine receptor antibody titer. Thymoma was excluded. The clinical response to corticosteroids, pyridostigmine and intravenous immunoglobulin was complete. EOM weakness was never observed during a 3-year follow-up, also during a later exacerbation of MG.     

Late onset myasthenia gravis

Late onset myasthenia gravis (MG) is defined as the onset of the disease after the age of 50 in a patient with no clinical or paraclinical evidence of a thymoma. Myasthenia gravis has a bimodal appearance for both sexes with a peak age of onset located in the early onset group and another peak for late onset of MG. Early onset of MG in men is approximately 10 years later than in females. In late onset MG, the thymus is smaller than in early onset and much of the lymphoid tissue has been substituted with fat. There is no morphological evidence that the thymus atrophy seen in late onset MG is any different from the thymus involution which is age-related. The AChR antibody concentration is lower in cases with late onset MG than it is in early onset and in thymoma-associated MG. Sera from late onset MG patients can be divided in 2 groups; those with and those without striated muscle (MGT 30) antibodies. Their presence is not an effect of age since they are not detected among old healthy individuals. Nor is it an effect of long MG duration, since there is no relation between titre and duration. Non-thymoma MG patients who demonstrate an immune response to titin identical to that observed in thymoma, have a worse prognosis and seem to do less well after thymectomy than late onset MG patients without muscle antiboides.     

Epidemiology and treatment of myasthenia gravis: a retrospective study using a large insurance claims dataset in Germany

Myasthenia gravis (MG) is a rare, chronic autoimmune disease with symptoms of fluctuating muscular weakness and fatigability. The aim of this retrospective cohort study was to estimate the prevalence and incidence of MG in Germany, and to understand the burden of disease and treatment patterns, based on anonymized German claims data. Two patient samples were identified: (1) incident MG patients with newly onset disease between 2015 and 2019, and (2) prevalent MG patients in 2019. In total, 775 incident MG patients with a mean age of 66.9 years; and 1,247 prevalent MG patients with a mean age of 68.6 years were included. The prevalence for Germany was estimated to be 39.3/100,000 on 31/12/2019; the incidence in 2019 was 4.6 cases/100,000 persons. The 12-month mortality was 5.7. For 31.5% of the incident patients, no MG treatment was observed in the first year after the index date. Of all incident patients, 29.9% experienced an exacerbation, and 6.7% a myasthenic crisis during the observation. Our study indicates that a substantial proportion of MG patients remains untreated. Many MG patients still experience exacerbations / MG crises. MG seems to be associated with an excess mortality in comparison to the general non-MG population.     

重症肌无力病人乙酰胆碱受体抗体的测定及临床意义

用ＥＬＩＳＡ（固相酶联免疫吸附）法测定１７２例ＭＧ病人血清乙酰胆碱受体抗体（ＡｃｈＲａｂ），结果显著高于健康献血员组和非ＭＧ病人组。不同性别、病程及临床类型与ＡｃｈＲａｂ无相关性，但４１～５０岁组的显著高于其他年龄组。６７例类固醇激素治疗组、２２例大剂量两种球蛋白治疗组、１２例胸腺切除术组及３例ＭＧ危象病人２４次血浆交换疗法（ＰＥ）组，治疗后伴随肌无力症状的好转，ＡｃｈＲａｂ均显著低于治疗前。结果表明：ＡｃｈＲａｂ测定为ＭＧ诊断提供了可靠的实验依据，为类固醇激素、大剂量丙种球蛋白、胸腺切除术和ＰＥ等治疗ＭＧ提供理论依据和疗效评定的实验指标，进一步证实了ＭＧ免疫学发病机理。     

幕上高血压脑出血早期血肿扩大的相关因素和预后分析

目的 探讨幕上高血压脑出血早期血肿扩大的相关因素和预后。方法 回顾性分析徐州医科大学附属医院神经外科2015年01月至2019年05月诊断为幕上高血压脑出血并入院时行保守治疗患者的临床资料,其中符合第四届全国脑血管病会议制定的诊断标准且有完整临床数据的患者共346人。按准入及排除标准,将其分为血肿扩大组（101人）及血肿未扩大组（245人）,分析患者性别、年龄、血型、是否有糖尿病史、血肿部位、血肿形态、血肿量、入院时格拉斯哥昏迷评分（GCS）、首次头颅CT距离发病时间、入院时红细胞分布宽度（RDW）、血红蛋白（HB）、血小板（PLT）、白细胞（WBC）、中心粒细胞（ANC）、淋巴细胞（ALC）、谷丙转氨酶（ALT）、谷草转氨酶（AST）、白蛋白（ALB）、肌酐（CREA）、血糖值（GLU）、血清钙（CA）、血清镁（Mg）、总胆固醇（CHOL）、甘油三酯（TG）、低密度脂蛋白（LDL）、高密度脂蛋白（HDL）、凝血功能（PT、APTT、FIB）、入院血压（SBP、DBP）等相关指标。根据患者发病后6个月的格拉斯哥预后分级来评估患者预后情况。结果 单因素分析显示血肿扩大组患者性别、血型、血肿形态、血肿量、首次CT距离发病时间、入院时格拉斯哥昏迷评分、血糖、低密度脂蛋白与血肿未扩大组之间差异有统计学意义（P<0.05）,进一步多因素Logistic回归分析显示患者性别、血型、血肿形态、首次CT距离发病时间、入院时格拉斯哥昏迷评分、血糖、低密度脂蛋白在两组患者之间差异有统计学意义（P<0.05）。预后结果显示血肿扩大组的患者死亡率和致残率明显高于血肿未扩大组患者。结论 患者性别、血型、血肿形态、首次CT距离发病时间、入院时格拉斯哥昏迷评分、血糖、低密度脂蛋白是幕上高血压脑出血早期血肿扩大的独立影响因素。发生血肿扩大的患者预后更差。     

Expression of Immune Molecules CD25 and CXCL13 Correlated with Clinical Severity of Myasthenia Gravis

Differential expressions of immune molecules have been shown in the thymi with pathological results, including myasthenia gravis (MG). CD25 is an activation marker expressed on T cells. CXCL13 mediates the homing and motility of B cells in secondary lymphoid tissues. Herein, we investigated the expressions of CD25 and CXCL13 in the thymi of thymic hyperplasia patients with MG or with non-MG. A total of 34 thymic hyperplasia patients with MG (20 generalized MG (GMG) and 14 ocular MG (OMG) and six thymic hyperplasia patients without MG were enrolled and analyzed using immunohistochemical staining and real-time polymerase chain reaction for CD25 and CXCL13. Our study demonstrated a higher expression of both CD25 and CXCL13 in hyperplastic thymi with OMG or GMG compared to those with non-MG. According to the immunohistochemical results, we observed that CD25 expression was significantly lower in atrophic thymi and non-MG hyperplastic thymi, compared with that in infant thymi (P?=?0.002 and 0.005, respectively). In contrast to CD25 expression, we did not observe differential expression of CXCL13 among three control groups. And a similar CD25 mRNA expression was found in real-time polymerase chain reaction (PCR) results. We observed that both hyperplastic thymi with OMG or GMG expressed significantly higher levels of CD25 than those with non-MG (P?=?0.007 and 0.001, respectively). And an increase of CD25 expression was observed in hyperplastic thymi with GMG compared to those with OMG (P?=?0.030). Similarly, CXCL13 expression was significantly higher in hyperplastic thymi with GMG or with OMG than those with non-MG (P?=?0.001 and 0.050, respectively). No significant CXCL13 expression difference was found between hyperplastic thymi with GMG and those with OMG (P?>?0.05). The real-time PCR results showed a similar tendency of CD25 mRNA expression among the thymi of non-MG, OMG, and GMG patients, but the difference did not reach significance (P?>?0.05). An obvious increased expression of CXCL13 was found in hyperplastic thymi with GMG patients, compared to those with non-MG and OMG patients (P?=?0.003 and 0.071, respectively). There was no difference found between hyperplastic thymi with non-MG and with OMG. Regression analysis showed a positive correlation between thymic CD25 level and MG symptom severity (F?=?28.240; P?=?0.000, r?=?0.523). Similarly, a positive correlation was found between thymic CXCL13 expression and MG disease severity (F?=?36.093; P?=?0.000, r?=?0.671). Taken together, our findings suggest CD25 and CXCL13 participate in the pathogenesis of MG and may influence the clinical symptoms of MG.