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1.
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。  相似文献   

2.
重症肌无力(myasthenia gravis,MG)是由乙酰胆碱受体抗体(acetylcholine receptor antibody,AChRAb)介导的、细胞免疫依赖的、补体参与的自身免疫性疾病,其主要临床症状为横纹肌收缩无力,多数患者起病表现为眼肌型MG(ocular MG,OMG),以后逐渐进展为全身型MG(generalizedMG,GMG),严重者累及呼吸肌,发生肌无力危象而死亡[1]。  相似文献   

3.
合并胸腺瘤的重症肌无力临床研究   总被引:2,自引:0,他引:2  
目的 探讨胸腺瘤病理分型、病理分期和重症肌无力(MG)之间关系。方法 分析1986-1996年88例经手术证实为胸腺瘤患者,其中28例术前合并MG,分析这28例患者胸腺瘤病理分型、病理分期与MG发生年龄、性别、Osserman临床分型及术后MG缓解加重关系特点。结果 发现胸腺瘤伴有MG28例(31.8%)。其中男18例占64.3%;女10例占35.7%;发病年龄以40-59岁高高峰期,共20例,占71.4%;病理分型证实上皮细胞型胸腺瘤MG发病率高,且临床症状重,以Osserman临床分型Ⅱa,Ⅱb型为主,肌无力危象发生率高,死亡率高;其次为上皮淋巴细胞混合型胸腺瘤,上皮细胞型胸腺瘤术后MG症状缓解后再加重多于混合型胸腺瘤,症状加重多见为上皮淋巴细胞混合型胸腺瘤,上皮细胞型胸腺瘤术后MG症状缓解后再加重比率高。结论 胸腺瘤伴MG患者以男性多见,发病年龄以40-59岁为主,以上皮细胞型为主,Masaoka病理分期为Ⅱ、Ⅲ期胸腺瘤MG临床症状重,且术后MG症状缓解后易再加重。  相似文献   

4.
目的探讨重症肌无力(myasthenia gravis,MG)患者汉密尔顿抑郁量表(Hamilton depression rating scale,HDRS)评分情况及其影响因素分析。方法横断面研究2013-07—2015-03作者医院就诊的188例MG患者的临床资料和HDRS评分情况,并根据HDRS评分将其分为抑郁组和非抑郁组,分析两组MG患者的临床特点及其与HDRS评分间的关系。结果所纳入MG患者男女比例为1.02∶1,眼肌型重症肌无力(ocular myasthenia gravis,OMG)和全身型重症肌无力(generalized myasthenia gravis,GMG)的比例为1.2∶1,以OMG起病和以GMG起病患者的比例为6.2∶1,病程中位数为2年,四分位数间距为1.8年,平均量化重症肌无力评分(quantitative myasthenia gravis,QMG)为(6.7±2.3)分,平均HDRS评分为(8.7±3.4)分,并发抑郁者65例,未并发抑郁者123例。影响HDRS评分和抑郁发生的相关因素包括性别(P0.01)、MG类型(P0.01)、QMG得分(P0.01)和美国重症肌无力协会(myasthenia gravis foundation of America,MGFA)分型(P0.01)、有无甲状腺功能亢进(P0.05)。结论影响MG患者HDRS评分和抑郁发生的相关因素包括性别、MG类型、QMG评分和MGFA分型、有无甲状腺功能亢进,充分认识其抑郁发生情况有利于更好地治疗MG。  相似文献   

5.
目的研究重症肌无力(MG)临床特点、误诊原因与MG危象临床表现。方法分析本院收治的169例MG患者的临床资料。结果本文患者均行新斯的明试验,阳性率100%。85例行重复神经电刺激(RNS)检查,64例低频波幅递减,8例同时高频波幅递减。157例行胸腺影像学检查,发现胸腺异常80例。发生肌无力危象31例次。121例行甲状腺功能(FT3、FT4、TSH)检测,发现甲状腺功能异常17例。结论MG临床表现多样,时有误诊,易于合并胸腺瘤或胸腺增生及甲状腺功能异常。肌无力危象发生率高。  相似文献   

6.
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。  相似文献   

7.
目的探讨柠檬酸提取物抗体(CAE-Ab)及烟碱型乙酰胆碱受体抗体(nAchR-Ab)测定对临床早期诊断伴发胸腺瘤的重症肌无力(MG)患者的价值,与改良Osserman分型及病理分型的关系.方法采用酶联免疫吸附法检测20例伴发胸腺瘤的重症肌无力患者的血清CAE-Ab、nAchR-Ab.结果 CAE-Ab阳性率75%,nAchR-Ab阳性率60%,大多在Ⅱa型与Ⅱb型患者.上皮细胞为主型4例中Ⅱb型3例、CAE-Ab均阳性,术后发生肌无力危象2例,1例死亡.结论 CAE-Ab及nAchR-Ab检测可作为临床诊断 MG患者伴发胸腺瘤的筛选检查;术后病理分型可指导治疗和判断预后.  相似文献   

8.
老年重症肌无力的临床特点(123例临床分析)   总被引:2,自引:0,他引:2  
目的 研究老年重症肌无力(MG)的临床特点。方法 回顾性分析1990-01—2002-05北京医院123例老年MG住院患者的临床特点,并与507例青壮年MG患者进行对比。结果 老年MG患者占同期MG患者的18.14%,男性患者较多(占68.29%),眼肌首发症状多见(占80.49%),分型以I型和Ia型多见(占56.10%),胸腺异常主要为胸腺瘤(占老年MG患者手术病理证实伴发胸腺异常的100%),低频重复电刺激(RNS)阳性率为74.19%,老年MG危象发生率较低(为2.44%)。结论 老年MG患者具有男性、眼肌首发症状、I型与IA型多见,以及胸腺异常主要为胸腺瘤,而RNS阳性率与MG危象发生率低等临床特点。  相似文献   

9.
目的探讨眼肌型重症肌无力(OMG)病情进展的影响因素及预测指标。方法回顾性分析了200例OMG患者的临床资料,根据病情进展情况分为OMG组和全身型重症肌无力(GMG)组两组,并对两组临床特征和实验窒检查结果进行比较。结果 GMG组乙酰胆碱受体抗体(AChRAb)水平升高、单纤维肌电图异常以及胸腺瘤比例均高于OMG组(P0.05)。Cox风险回归模型分析结果显示早期给予糖皮质激素和免疫抑制剂治疗能明显降低GMG发生的风险(分别RR=0.377、RR=0.193,均P0.01),而AChRAb水平升高和合并胸腺瘤可以增加GMG发生的风险(分别RR=4.460、P0.01,RR=2.113、P0.05)。结论 AChRAb水平升高、合并胸腺瘤可促进OMG病情进展,有可能作为OMG进展为GMG的风险预测指标。早期糖皮质激素治疗以及长期口服免疫抑制剂治疗有可能延缓OMG病情向GMG进展。  相似文献   

10.
胸腺瘤病理与重症肌无力   总被引:1,自引:1,他引:0  
目的 研究胸腺瘤病理组织分型,胸腺瘤Masaoka临床分期和重症肌无力(MG)发生率,以及Osserman分型与预后之间的关系。方法 回顾性分析1989-1999年间79例胸腺瘤术后病理所见与临床特点。结果 79例胸腺瘤患者中伴MG者25例(31.8%)。上皮细胞型29例,并发MG12例(41.4%);按MG的Osserman分型。Ⅰ型1例(8.3%),Ⅱa型3例(25%),Ⅱb型5例(40.8%),Ⅲ型3例(25%),术后出现肌无力危象2例(16.7%),死亡1例(8.3%)。淋巴细胞型14例,并发MG2例(14.3%);按MG的Osserman分型,Ⅰ型1例(7.1%),Ⅱa型1例(7.1%)。混合细胞型36例,并发MG11例(30.6%);按MG的Osserman分型,Ⅰ型2例(18.2%),Ⅱa型1例(9.1%),Ⅲ型4例(36.4%),Ⅳ型1例(9.1%);术后出现肌无力危象1例(9.1%),死亡1例(9.1%),其他类型胸腺瘤25例,按MG患者的Masaoka临床分期;Ⅰ期3例(12%),Ⅱ期4例(16%),Ⅲ期12例(30%),Ⅳ期6例(24%);Ⅰ、Ⅱ期以Osserman分型Ⅰ型、Ⅱ型为主,Ⅱ、Ⅳ期以Osserman分型Ⅲ型,Ⅳ型为主,25例MG中18例术后临床症状得到明显改善(72%)。结论 胸腺瘤病理组织分型可能与MG的发生,Masaoka临床分期和Osserman分型及预后有关。  相似文献   

11.
目的 :探讨眼肌型重症肌无力进展为全身型重症肌无力的临床相关预测因素。方法 :33例初诊为眼肌型重症肌无力的患者经过3年随访,根据疾病进展结局分为眼肌型重症肌无力组(13例)和进展为全身型重症肌无力组(20例)。对与疾病进展可能相关的临床因素进行分析。结果 :进展为全身型重症肌无力组患者初诊时的定量重症肌无力评分、乙酰胆碱受体抗体阳性率、抗核抗体阳性率、合并胸腺瘤的比例以及合并糖尿病的比例均高于眼肌型重症肌无力组(P值均0.05)。结论 :定量重症肌无力评分高、乙酰胆碱受体抗体阳性、抗核抗体阳性以及合并胸腺瘤和糖尿病可能是眼肌型重症肌无力进展为全身型重症肌无力的预测指标。  相似文献   

12.

Background and Purpose

The World Health Organization (WHO) has classified thymic carcinoma and other thymomas (types A, AB, and B) as different neoplasms. Myasthenia gravis (MG) is an early sign of thymoma and theoretically does not accompany thymic carcinoma; however, cases of thymic carcinoma with MG have been reported. Whether thymic carcinoma can accompany MG has yet to be established.

Methods

The medical records of patients who underwent thymectomy for MG between 1990 and 2011 in a single hospital were reviewed. All cases with the diagnostic code of "thymic carcinoma" or "thymoma type C" (old terminology) were selected. A pathologist re-reviewed the pathologic specimens using the new WHO criteria. The rate of thymic carcinoma among these MG patients was then calculated.

Results

A total of 81 patients with MG had thymic tumors, 10 of whom had thymic carcinomas or thymoma type C. Seven cases of well-differentiated thymic carcinomas (type B3) were excluded, leaving three (3.7%) cases of thymic carcinoma with MG. All three of these cases were type B3 thymoma with a focal squamous cell carcinoma component that was very small and well demarcated. In addition, two out of the three tumors were found to be at an early clinical stage. All of the cases survived without recurrence over follow-up periods of at least 5 years.

Conclusions

Thymic carcinoma transformation from thymoma can occur during the early stages of thymoma. The association of this condition with MG is not as rare as was previously thought. Thymic carcinomas accompanying MG had a predominant B3 thymoma component with a focal thymic carcinoma area (squamous cell carcinoma).  相似文献   

13.
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.  相似文献   

14.
目的探讨伴有不同胸腺情况的重症肌无力(MG)患者对药物及胸腺切除术(TX)的疗效。方法对我院确诊的71例MG患者进行回顾性分析,根据胸腺CT情况将患者分为胸腺CT正常组、合并胸腺增生组、合并胸腺瘤组及合并胸腺癌组,采用Fisher精确检验法比较各组对两种治疗方法的疗效。结果胸腺CT正常的MG患者多采取药物治疗,缓解率为52%,有效率为88.5%;合并胸腺增生、胸腺瘤、胸腺癌的MG患者多采取了TX治疗。合并胸腺增生组的术后缓解率(62.5%)明显高于合并胸腺瘤组(8.3%)(P<0.05);合并胸腺增生组的术后有效率(93.7%)与合并胸腺瘤组差异无显著性(83.3%)(P>0.05)。结论合并胸腺增生的MG患者TX的术后疗效优于合并胸腺瘤者。  相似文献   

15.
眼肌型重症肌无力临床分析   总被引:1,自引:0,他引:1  
目的总结分析眼肌型重症肌无力患者的临床特征,以为诊断和治疗提供参考依据。方法回顾性分析113例眼肌型重症肌无力患者的临床资料。采用免疫荧光细胞染色方法检测血清乙酰胆碱受体(AChR)抗体和肌肉特异性受体酪氨酸激酶(MuSK)抗体表达水平,分析这两项免疫学指标对眼肌型重症肌无力向全身型转化的预测价值。结果成年发病的眼肌型重症肌无力好发于40岁以上男性,多以眼睑下垂(95例,84.07%)为首发症状,少数以复视(18例,15.93%)起病。疲劳试验和新斯的明试验阳性率分别为79.44%(85/107)和84.85%(84/99).低频重复神经电刺激和血清甲状腺抗体异常率分别为44.32%(39/88)和28%(14/50),胸腺增生和胸腺瘤阳性率分别为16.67%(17/102)和11.76%(12/102);血清AChR抗体阳性率为62.83%(71/113);但MuSK抗体均呈阴性。眼肌型重症肌无力向全身型转化率为12.39%(14/113),其中血清AChR抗体强阳性者(13例,28.26%)显著高于弱阳性者(1例,4%),二者差异有统计学意义(X^2=4.587,P=0.032)。结论成年发病的眼肌型重症肌无力好发于中年以上男性,主要表现为眼睑下垂和复视,大多数患者伴发胸腺和甲状腺异常。血清AChR抗体表达水平升高预示向全身型转化率升高,鲜有MuSK抗体阳性反应。  相似文献   

16.
目的比较非胸腺瘤性眼外肌麻痹起病重症肌无力(ocular onset myasthenia gravis,OMG-O)患者于不同病程行胸腺切除后病情缓解与向全身型重症肌无力(GMG)转化的差异,以期对手术时机进行探讨。方法采用双向队列研究方法,对作者医院2005年1月至2017年9月因药物治疗效果不佳而行胸腺切除的86例OMG-O患者,按起病至手术时间间隔分为早手术组(≤1年)、晚手术组(>1年),比较两组患者不同随访时间理想状态(干预后状态达到轻微异常及更好)率、GMG转化率的差异。结果 (1)与早手术组比较,晚手术组少年MG(<18岁)比例较高,起病至手术时间间隔、起病至使用激素时间间隔、激素用药时间较长(均P <0.05)。(2)与晚手术组比较,早手术组术后第3年理想状态率高(P=0.029),而第2、4~8年理想状态率有升高趋势,但差异无统计学意义(P>0.05)。(3)9例术后发生GMG转化,起病至发生GMG转化时间中位数17.8个月,其中6例(66.7%)在3年内发生GMG转化。早手术组与晚手术组间GMG转化率比较,差异无统计学意义(12.5%比8.7%;χ~2=0.049,P=0.825)。结论对于药物治疗无效或不耐受的OMG-O患者,早期行胸腺切除手术疗效较好;但即使手术,仍有可能发生GMG转化,约1/2患者于起病后1.5年内发生,约2/3于起病后3年内发生。  相似文献   

17.
Extraocular muscle weakness is the most common presenting sign of myasthenia gravis (MG). More than half of patients presenting with symptoms isolated to these muscles (OMG) develop generalized myasthenia gravis (GMG) over the course of their illness. No clinical, laboratory, or electrophysiological features are recognized that identify these high-risk patients. We have therefore assessed the ability of single-fiber electromyography (SFEMG) to predict the development of GMG in patients presenting with OMG. Thirty-nine consecutive patients presenting with OMG underwent SFEMG of the extensor digitorum communis muscle as well as a battery of other laboratory and imaging studies at the time of diagnosis. All patients were followed prospectively for a minimum of 24 months or until they developed GMG. Two patients were excluded, leaving 37 for assessment. Twenty remained with pure OMG for the entire follow-up period (mean, 55 months). Twenty-six of the 37 had abnormal SFEMG studies at presentation. Eleven of these remained with OMG and 15 developed GMG. Fifty-eight percent of patients with an abnormal SFEMG developed GMG, whereas 82% of those with a normal study remained with OMG. Thus, a normal SFEMG was associated with MG remaining restricted to the extraocular muscles. (P = 0.036, Fisher's exact test), but an abnormal SFEMG was not predictive of subsequent development of GMG.  相似文献   

18.
69例重症肌无力(MG)病人行胸腺切除术,术前血清乙酰胆碱受体抗体水平均高于正常,术后半年抗体水平明显下降,胸腺增生组(20例)尤为显著.胸腺瘤组(49例)术前胸腺瘤相关抗体水平增高,但术后半年内无明显变化.69例MG患者胸腺切除术后4年,症状总缓解率为76.81%,其中胸腺增生组95%,胸腺瘤组69.39%。作者认为,对于伴有胸腺增生或胸腺瘤的MG患者,胸腺切除术是有效的,应该列为治疗的第一选择。  相似文献   

19.
目的总结分析眼肌型重症肌无力患者的临床特征,以为诊断和治疗提供参考依据。方法回顾性分析113例眼肌型重症肌无力患者的临床资料。采用免疫荧光细胞染色方法检测血清乙酰胆碱受体(AChR)抗体和肌肉特异性受体酪氨酸激酶(MuSK)抗体表达水平,分析这两项免疫学指标对眼肌型重症肌无力向全身型转化的预测价值。结果成年发病的眼肌型重症肌无力好发于40岁以上男性,多以眼睑下垂(95例,84.07%)为首发症状,少数以复视(18例,1 5.93%)起病。疲劳试验和新斯的明试验阳性率分别为79.44%(85/107)和84.85%(84/99),低频重复神经电刺激和血清甲状腺抗体异常率分别为44.32%(39/88)和28%(14/50),胸腺增生和胸腺瘤阳性率分别为16.67%(17/102)和11.76%(12/102);血清AChR抗体阳性率为62.83%(71/113);但MuSK抗体均呈阴性。眼肌型重症肌无力向全身型转化率为12.39%(14/113),其中血清AChR抗体强阳性者(13例,28.26%)显著高于弱阳性者(1例,4%),二者差异有统计学意义(X~2=4.587,P=0.032)。结论成年发病的眼肌型重症肌无力好发于中年以上男性,主要表现为眼睑下垂和复视,大多数患者伴发胸腺和甲状腺异常。血清AChR抗体表达水平升高预示向全身型转化率升高,鲜有MuSK抗体阳性反应。  相似文献   

20.
目的探讨乙酰胆碱受体抗体(AChR-Ab)与重症肌无力(MG)临床特征的相关性。方法采用放射免疫法检测115例MG患者及92例对照组(非MG神经系统疾病患者42例,健康体检者50名)血清AChRAb浓度,应用临床绝对评分记录MG患者病情严重程度。分析各组血清AChR-Ab浓度的差异,以及AChR-Ab浓度与MG患者临床特征的相关性。采用ROC工作特征曲线探讨AChR-Ab诊断MG的敏感度和特异度。结果MG患者血清AChR-Ab浓度中位数(四分位数间距,下同)为3.45(39.38)nmol/L,较非MG神经系统疾病患者[0(0)nmol/L]和健康体检者[0(0)nmol/L]增高(P0.01)。全身型MG(GMG)患者AChR-Ab浓度[25.45(46.14)nmol/L]较眼肌型MG(OMG)患者[0.58(3.56)nmol/L]增高(P0.01)。用ROC曲线法分析显示,以血清AChR-Ab浓度≥0.50nmol/L作为诊断MG界值时灵敏度为72.17%,特异度为100%,曲线下面积(AUC)=0.895(95%CI:0.849~0.941)。AChR-Ab浓度与发病年龄、病程及改良Osserman分型呈正相关(r=0.220,P0.05;r=0.184,P0.05;r=0.382,P0.01),但相关性较弱(均r0.5),与临床绝对记分无相关性(r=0.147,P0.05)。结论用放射免疫法检测血清AChR-Ab浓度诊断MG的灵敏度和特异度均高,有助于减少MG的漏诊率及误诊率,值得临床推广。  相似文献   

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