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。     

老年期重症肌无力的临床特点

目的 研究老年期重症肌无力（ＭＧ）的临床特点，方法 将２０年间诊治的１９２４例ＭＧ，根据发病年龄分为小儿期ＭＧ（≤１４岁）成年期ＭＧ（１５～５９岁）及老年期ＭＧ（≥６０岁），采用Ｘ＾２检验比较３组的临床资料。结果 老年期ＭＧ在整个ＭＧ中构成比小（只占５．５％）男性发病者较多见（男：女＝１．４：１）以延髓症状首发者多见，合并胸腺者多而全并其他自体免疫病者少，以延髓症处发面误诊为脑血管者多见，病死率高     

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。     

女性重症肌无力1316例临床特点分析

目的　研究女性重症肌无力 (MG)的临床特点及某些实验室指标。方法　回顾性分析1984～ 2 0 0 2年我院诊治的 1316例女性MG患者的临床资料 ,检测乙酰胆碱受体抗体和血清性激素水平 ,并与同期诊治的男性MG患者进行对比分析。结果　MG患者男女比例基本相同 (1∶1 0 1) ,但不同年龄段男女患者比例差异有显著意义 ;延髓肌型MG虽少见 ,但女性 (6 0例 )患者明显多于男性 (35例 ,P <0 0 5 ) ;女性MG患者病情加重或者发生危象存在特殊诱因 ;女性MG患者伴胸腺瘤者较男性低 ;女性MG患者合并其他自身免疫性疾病者更多 ;MG患者存在性激素水平异常 ,女性较男性为甚。结论　我国女性MG的临床特点与男性MG相比有其独特之处 ,充分认识这些特点将有利于指导临床的诊断和治疗。     

重症肌无力80例临床分析

目的 探讨重症肌无力患者的临床特点、发病情况及治疗效果.方法 回顾性分析了80例重症肌无力患者的发病年龄、发病诱因、临床表现、合并症及治疗效果.结果 重症肌无力患者发病年龄平均(32.5±10.8)岁,男女之比为1.1:1.发病的诱因以感染为首位,80例病人均采用药物治疗,总有效率达97.5%.有35 %的病人合并有胸腺瘤、胸腺增生,行胸腺切除术.结论 重症肌无力临床表现复杂多样,药物及胸腺切除治疗均有效.预防及控制感染是防止重症肌无力反复发作,减少重症肌无力危象的有效方法.     

重症肌无力19例临床分析

目的方法对19例重症肌无力(MG)患者的临床特点和治疗效果进行回顾性分析、总结临床经验。结果男性患者8例,女性患者11例,男女比例1:1.375。发病年龄为2~77岁,平均年龄40.63岁。首发症状为眼睑下垂者10例,占52.6%;其中眼睑下垂合并锥体束征1例,合并甲状腺功能亢进者4例,合并右耳听力下降者1例。19例患者均在应用抗胆碱酶药物溴吡斯的明基础上加用激素或丙种球蛋白治疗,其中有效16例,无效3例,总有效率为84.2%。合并胸腺瘤者5例,均行胸腺切除,术后有效4例,无效1例,总有效率80%。结论 MG多青壮年发病,10%~15%合并胸腺瘤,临床表现复杂多样,首发症状以眼睑下垂最为多见,激素、丙种球蛋白及胸腺切除术均为治疗MG的有效手段。     

伴甲状腺疾病重症肌无力患者的临床特点

目的 研究伴甲状腺疾病的重症肌无力(MG)患者的临床特点.方法 同期住院及门诊MG患者88例(均排除其他自身免疫性疾病),分为甲状腺正常组(61例)和伴甲状腺疾病组(27例),回顾性分析其资料,比较两组患者的一般情况、首发症状、临床分型、实验室检查、治疗及疗效等临床特点.结果 伴甲状腺疾病MG组中女性比例明显高于甲状腺...     

伴胸腺瘤重症肌无力的临床特点(附96例分析)

目的　研究伴胸腺瘤重症肌无力的临床特点。方法　对经手术病检证实的 96例MG伴胸腺瘤患者的临床资料进行回顾性分析 ,并采用 χ2 检验及t检验与 114 9例经影像学检查无胸腺瘤表现的MG患者进行比较。结果　胸腺瘤组男性发病者多 (男∶女 =1 82∶1) ,且多于 30岁后发病 (71 9% ) ,以肢体无力和延髓症状首发者多见 (4 3 8% ) ,按改良Os serman分型 ,以Ⅲ型和Ⅳ型为主 (4 5 8% ) ,危象发生率高 (38 5 % ) ,病死率高 (8 3 % )。结论　伴胸腺瘤的重症肌无力有其独特临床特点 ,充分认识这些特点将有利于指导对这类患者的临床诊断和治疗     

不典型眼肌型重症肌无力的临床特点

目的研究不典型眼肌型重症肌无力(OMG)的临床特点。方法回顾性分析29例不典型OMG患者的临床资料。结果本组患者表现为上眼睑下垂3例,单眼1条眼外肌瘫痪15例,双眼3条眼外肌瘫痪1例,单眼全部眼外肌瘫痪1例,辐辏障碍2例,单眼眼轮匝肌瘫痪1例,类似眼病表现6例(复视4例、眼部不适和视物模糊2例,均无眼外肌瘫痪);有晨轻暮重表现12例(41.4%)。新斯的明试验阳性26例(89.7%),低频重复神经电刺激阳性8例(27.6%),单纤维肌电图(SFEMG)异常23例(79.3%)。患者早期均被误诊。经泼尼松和/或胆碱酯酶抑制剂治疗,症状消失26例(89.7%),显著改善3例(10.3%)。结论不典型OMG患者的临床特点为受累眼肌少,症状局限,表现晨轻暮重的比率低。新斯的明试验和SFEMG检查可确诊。     

非胸腺瘤性眼外肌麻痹起病重症肌无力胸腺切除时机探讨

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。     

Juvenile myasthenia gravis with prepubertal onset

Juvenile myasthenia gravis (JMG) with prepubertal onset is an uncommon disease. We studied 19 patients with age at onset ranging from 1.5 to 9.2 years and compared their clinical characteristics and response to therapy with 114 cases with MG onset after the prepubertal age, up to 20 years. Neither sex prevalence nor autoimmune diseases other than MG were found in younger patients. Although ocular myasthenia was more frequent than in later-onset JMG, children with generalized symptoms were often severely affected and respiratory involvement was present in 8/19 patients. Anti-acetylcholine receptor antibodies were detected at a lower rate and, in contrast with results in older patients, seronegativity was more frequent among children with generalized disease. Three out of six patients with onset before the age of five showed spontaneous remission. Nine prepubertal patients underwent thymectomy and, as most of them also received immunosuppressive therapy, the influence of surgery on disease outcome remains unclear; in no case was thymoma found. This is in contrast to the good results after thymectomy and the presence of thymoma in the later-onset group. Eleven patients in the prepubertal series were treated with immunosuppressive therapy. At the end of follow-up, most patients were in good condition. The frequency of immunosuppressive therapy and the rate of good therapeutic results did not differ from those observed in older patients.     

白细胞介素-6与重症肌无力患者临床特点的关系

目的　探讨白细胞介素 6(IL 6)与重症肌无力(MG)患者临床特点的关系。方法　采用双抗体 夹心ELISA法检测36例MG患者及20名健康对照者的血清IL 6和乙酰胆碱受体抗体(AchRAb)水平,并分 析其与MG临床特点的关系。结果　MG患者血清IL 6水平高于健康对照者(P<0.01);AchRAb阳性患者 高于阴性患者(P<0.05);全身型患者高于眼肌型患者(P<0.05);病情重者高于病情轻者(P<0.05);急性 期高于非急性期(P<0.01);预后差者高于预后好者(P<0.05);伴胸腺异常者高于胸腺正常者(P<0.05)。 结论　IL 6与MG临床特点相关,在MG发病机制中起重要作用。血清IL 6水平可间接反映体内免疫功能紊 乱的程度,对判断MG患者病情、预后和指导治疗有重要的参考价值。     

Studying the relationship between clinical features and mental health among late-onset myasthenia gravis patients

BACKGROUNDMental disorders are common comorbidities among individuals with neurological diseases, and the prevalence of depressive and anxiety-related symptoms in newly referred patients at neurology outpatient clinics is high. There have been few studies on the mental health of patients with late-onset myasthenia gravis (MG).AIMTo examine the relationship between clinical features and the mental health symptoms within late-onset MG patients.METHODSA total of 105 patients diagnosed with MG were recruited consecutively from a neuromuscular outpatient clinic between December 2020 and February 2021. Patients were classified into two groups: early-onset MG (age at onset < 50 years, n = 63) and late-onset MG (age at onset ≥ 50 years, n = 42). Social demographic data and information about marital status, education level, clinical symptoms, serum antibody levels, and therapies used were collected for all participants. Participants were also evaluated using the Myasthenia Gravis Composite scale, the Myasthenia Gravis Activities of Daily Living scale, the Myasthenia Gravis Quality of Life 15 (MG-QOL-15) questionnaire, the 17-item version of the Hamilton Depression Rating Scale (HAM-D) and the Hamilton Anxiety Rating Scale (HAM-A). The relationship between clinical features and mental health in late-onset MG patients was examined using multivariate logistic regression analyses. RESULTSLate-onset MG patients were more prone to dyspnea, had higher levels of serum anti-acetylcholine receptor antibodies, and higher total scores on the MG-QOL-15, HAM-D, and HAM-A questionnaires, than early-onset MG patients had (P < 0.05). Among those with late-onset MG, female patients had higher total HAM-D and HAM-A scores than male patients had (P < 0.05). High scores on the QOL-15 questionnaire were associated with higher incidences of anxiety and depression, and the association was found to be independent after adjusting for confounding risk factors. In the late-onset subgroup, the areas under the receiver operating characteristic curves for the MG-QOL-15 score-based diagnostic accuracy for anxiety and depression state were 0.816 (P = 0.001) and 0.983 (P < 0.001), respectively.CONCLUSIONHigher MG-QOL-15 scores were a risk factor for anxiety and depression in late-onset MG, and women with late-onset MG were more likely to have anxiety and depression than men were.     

Comparison of diagnosis value for new onset myasthenia gravis by many clinical auxiliary examinations

BACKGROUND: The clinical values of neostigmine test, clinical electrophysiologic study and acetylcholine receptor antibody detection in diagnosing myasthenia gravis (MG) found newly are unclear in China. OBJECTIVE: To investigate the reference value of common clinical diagnosis parameters in correctly diagnosing untreated MG found newly. DESIGN: Retrospective case analysis. SETTING: Department of Neurology, Beijing Hospital, Ministry of Health. PARTICIPANTS: Totally 156 outpatients with MG admitted to Department of Neurology, Beijing Hospital, Ministry of Health between January 1999 and December 2002. The involved patients, 72 males and 84 females, were aged 2–79 years. They were classified according to Osserman's criteria: ⅡA 72，ⅡB 76, Ⅲ 3 and Ⅳ 5. They were all subjected to being inquired of disease history, neostigmine test, and acetylcholine receptor antibody detection, met the diagnosis criteria of Neuroimmunology Committee of China, and confirmed by clinical electrophysiologic detections; Informed consents were obtained from all the involved subjects. METHODS: ①After admission, every patient was intramuscularly injected with 1.5 mg neostigmine; If the patient was a child, the injection dose was decreased according to his/her age. If his/her score of any observation index after injection was improved ≥ 50% as compared with before injection , his positive index was set as positive. Positive neostigmine test was set if there was one positive index. ②Repetitive nerve stimulation and single fiber electromyography were performed with Dantec Keypoint electromyogram (EMG) apparatus. ③Acetylcholine receptor antibody was detected by ELISA method. MAIN OUTCOME MEASURES: Clinical absolute and relative scores of MG, acetylcholine receptor antibody level, and repetitive nerve stimulation and single fiber electromyography examination results. RESULTS: The positive rates of neostigmine test, repetitive nerve stimulation and single fiber electromyography examination for MG were 86.5%, 82.6%, and 69.2%, respectively, and the positive rate of acetylcholine receptor antibody was 78.8%. CONCLUSION: Standardized neostigmine test has the highest sensitivity to diagnose MG.     

Plasma exchange in myasthenia gravis: clinical effect

Seventeen patients suffering from myasthenia gravis were treated with plasma exchange. Serious complications were not encountered. The effect usually appeared on the second or the third day of treatment. In 12 patients with a total of 31 plasma exchange courses, the treatment resulted in a shift in Osserman group. Six of these had long-standing remissions. Three patients responded moderately and two patients did not respond to plasma exchange.