晚发型重症肌无力的研究进展(综述)

重症肌无力 ( MG)在老年人口中发病率上升。流行病学资料支持以 5 0岁来区分早发 MG与晚发 MG。早发 MG与晚发 MG主要的免疫学差异是存在抗连接素的自身抗体 ,可在约 5 0 %的晚发 MG患者的血清中探测到。晚发 MG的治疗需针对患者的年龄和其特殊的免疫学表现两方面。     

重症肌无力患者血清干扰素-α抗体的检测意义

目的研究重症肌无力（MG）患者外周血中干扰素α抗体（IFN-αAb）的含量,并探讨其与MG的关系。方法采用ELISA法测定60例MG患者、20例正常对照组（NC）及20例非MG其他神经系统疾病患者（OND）血清中IFN-αAb。结果 发现伴胸腺瘤的重症肌无力患者（MGT）血清中IFN-αAb阳性率为75%,明显高于不伴胸腺瘤的重症肌无力患者（NTMG,11.5%）及对照组（P〈0.05）;晚发型MG者IFN-αAb阳性率为29.41%,明显高于早发型MG患者7.69%（P〈0.05）。结论 伴胸腺瘤的MG患者及晚发型MG患者外周血中IFN-αAb表达增高。     

晚发型重症肌无力的临床特点(附48例分析)

<正> 重症肌无力(MG)是自身免疫性疾病,近60％,患者在20～40岁之间发病。50岁以后发病的(晚发型)很少见。 资料与方法 1.一般资料:观察2001～2002年住院的98例MG病人,其中晚发型(≥50岁)的病人48例(48.9％,48/98),平均年龄62.5±13.6岁(50～79岁);男性27例,女性21例;病程15天～21年,平均2.5年。     

晚发型重症肌无力的胸腺切除

重症肌无力(MG)的治疗在文献中已有广泛报导,但对没有胸腺瘤的MG 采用类固醇治疗和胸腺切除的效果尚无对照研究,特别是对老年人的MG尚有许多问题有待解决。本文报道2例晚发型MG 采用胸腺切除获得成功。例1:男61岁,1981年有复视,颌无力,讲话及吞咽困难,肛门括约肌无力。腾喜龙试验阳性(手     

合并胸腺瘤的重症肌无力临床研究

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。     

晚发型偏头痛与青年偏头痛临床特点的比较

目的 比较晚发型偏头痛和青年偏头痛患者的临床特点.方法 对40例晚发型偏头痛患者(晚发型组)和40例青年偏头痛患者(青年组)的临床资料进行收集和比较.结果 晚发型组19例(47.5％)发病有明显诱因,青年组3例(7.5％)有明显诱因,差异有统计学意义(P＜0.05).晚发型组中先兆型偏头痛6例(15.0％),无先兆型33例(82.5％),偏瘫型1例(2.5％);青年组先兆型偏头痛15例(37.5％),无先兆型25例(62.5％).晚发型组中先兆型比率显著低于青年组(P＜0.05).晚发型组单侧头痛及额部头痛的比率及头痛程度显著低于青年组,双侧头痛及全头痛的比率显著高于青年组(均P＜0.05).晚发型组伴面色苍白、厌食及口干的比率显著高于青年组(均P＜0.05).晚发型组头痛性质、持续时间、发作频率及缓解因素与青年组比较,差异无统计学意义.结论 与青年偏头痛相比,晚发型偏头痛患者发作有诱因的比率高,出现先兆症状少,头痛程度轻,多为双侧及全头痛;易合并自主神经症状.     

重症肌无力合并胸腺瘤的外科治疗

采用切除胸腺瘤治疗各型重症肌无力(MG)46例,病理证实胸腺瘤15例,恶性胸腺瘤31例,伴异位胸腺增生1例。术后52.2％的病例发生了MG危象,其危象见于Ⅱ_B和Ⅲ型病例。10.9％的病例在院死亡。术后讨39例病人进行了6个月～12年的随访,症状缓解33.3％,改善20.5％,无变化2.6％,与本病有关的死亡者为38.5％,MG合并胸腺瘤病例术后MG危象发生率高,围手术期和离院后死亡率亦较高。近年来对病情危重病例胸腺瘤切除早期行气管切开,酌情辅助呼吸,是预防和治疗MG危象的重要方法,有助于降低围手术期死亡率。     

晚发型维生素K1缺乏症致颅内出血34例临床治疗体会

目的 总结晚发型维生素K1缺乏症致颅内出血临床治疗体会。方法 回顾性分析2012年1月至2015年10月收治的34例晚发型维生素K1缺乏症致颅内出血的临床资料。脑室周围-脑室内出血7例及单纯蛛网膜下腔出血3例,反复腰椎穿刺术脑脊液置换后病情好转。脑室周围-脑室内出血合并脑室扩大、梗阻性脑积水4例,紧急行储液囊脑室外引流术。硬膜下出血10例中,5例保守治疗;5例伴占位效应、中线结构偏移,急诊手术治疗。脑内血肿10例中,7例保守手术;3例血肿量较大,行开颅血肿清除术。结果 存活32例,放弃治疗2例;存活32例中,25例神经功能改善良好,7例改善差。治疗后随访3个月~1年,预后良好22例,预后中等7例,预后差3例。结论 晚发型维生素K1缺乏症致颅内出血往往病情危重,应早期诊断、及时治疗,对于不同出血类型采用个体化治疗方案,实施个体化的围手术期管理,可有效改善预后。     

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。     

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.     

Characteristics of late-onset myasthenia gravis

An increasing incidence of myasthenia gravis (MG) has been reported in the elderly, but the full clinical ramifications of late-onset myasthenia gravis (LOMG) remain unclear. We describe the clinical features of our cohort of patients with MG with an emphasis on an onset after the age of 50. This was a retrospective analysis of medical records of a cohort of patients followed in two tertiary neuromuscular clinics and comparison of early onset MG (EOMG) versus LOMG. There were 174 patients with a mean age of onset of 55.2?±?19.1?years, and 44 % were women. Late onset of myasthenia gravis after age 50 was reported in 114 patients (66 %). Anti-AChR antibody titers were elevated in 78 % of patients (65 % with EOMG vs. 85 % with LOMG; p?=?0.003), and frequency of elevated titers of anti-MuSK antibodies was similar in both groups (present in 38 % of all tested seronegative patients). Myasthenic crisis was equally common in generalized EOMG and LOMG (13 %). Ocular MG was more common in LOMG compared to EOMG (40 vs. 18 %, p?=?0.021). Diabetes was more prevalent with LOMG (27 vs. 5 %; p?=?0.0002). Overlapping clinical features of EOMG and LOMG are consistent with a continuous clinical spectrum of a single condition, with more frequent occurrence of seropositive and ocular MG with a late onset. A higher burden of comorbidities, such as diabetes mellitus, may warrant a modified approach to treatment of myasthenia in LOMG. However, overall disease severity may not be higher with aging. These observations have implications for design of MG clinical trials and outcomes studies.     

Thymectomy in late-onset myasthenia gravis

Two cases of late-onset myasthenia gravis were successfully treated by thymectomy using a sternal splitting technique, in spite of the fact that no thymomas could be detected preoperatively. One patient was seriously ill, the other patient responded to medical treatment. Thymolipoma and malignant thymoma, respectively, were removed from the patients. It is stressed that not all thymomas produce antibodies to striated muscles and that CT-scan of the mediastinum is of limited value in the diagnosis of thymoma. It is suggested that patients with late-onset myasthenia gravis be offered thymectomy, even in the absence of detectable thymomas.     

Thymectomy for myasthenia gravis: a fourteen-year experience

Over the last 14 years we have thymectomized 106 myasthenic patients, 62 by transcervical and 44 by transsternal approach. At the end of the follow-up 49 patients (46,2%) had improved, 12 of these (11,2%) being in remission. Our data supply no guidance regarding the prognosis of individual patients at the time of thymectomy. In our hands neither operation seemed to modify the natural course of myasthenia gravis.

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Sommario Nel corso degli ultimi 14 anni abbiamo sottoposto a timectomia 106 miastenici, 62 di questi per via transcervicale e 44 per via transsternale. Abbiamo ottenuto un miglioramento complessivo in 49 pazienti (46.2%); 12 di questi (11.2%) sono risultati in remissione completa. In nessuna delle sottopopolazioni di miastenici, da noi esaminate, è stato possibile evidenziare una precisa indicazione prognostica per la timectomia. Sembra di poter concludere che la timectomia, nei 2 approcci da noi utilizzati, non modifichi il decorso naturale della miastenia gravis.

     

阿立哌唑治疗晚发性分裂症的临床研究

目的 探讨阿立哌唑治疗晚发性分裂症的临床疗效和不良反应.方法 将符合CCMD-3诊断标准的72例首发未服药的晚发性分裂症患者随机分为两组,分别给予阿立哌唑和奋乃静治疗,疗程8周;采用简明精神病评定量表(BPRS)于治疗前后评定疗效,治疗中出现的不良反应量表(TESS)评定不良反应.结果 阿立哌唑与奋乃静疗效无显著性差异,阿立哌唑引致的锥体外系反应显著低于奋乃静.结论 阿立哌唑、奋乃静治疗晚发性分裂症的疗效相当,前者不良反应轻,安全性高.     

早发和晚发型强迫症住院患者临床特征及发病年龄的影响因素

背景 强迫症病因复杂且临床表现异质性高,不同年龄段起病的强迫症患者在临床症状及病因学上存在差异,目前针对早发型和晚发型强迫症住院患者的研究有限。目的 探讨早发型和晚发型住院强迫症患者临床特征的差异及影响强迫症发病年龄的因素,为强迫症患者的早期筛查及治疗提供参考。方法 收集2012年3月-2023年3月在南京医科大学附属脑科医院住院治疗的540例强迫症患者的病历资料。以起病年龄18岁为界,将患者分为早发组（n=310）和晚发组（n=230）,比较两组患者人口学资料及临床症状的差异。使用二元Logistic回归分析影响强迫症患者发病年龄的因素。结果 人口学资料方面,早发组和晚发组的性别、婚姻状况、精神疾病家族史、共病精神疾病、工作情况、受教育程度、强迫症状种类差异均有统计学意义（χ²=22.302、170.556、9.224、13.624、242.277、59.791、7.231,P均<0.05或0.01）,两组起病年龄、住院时年龄差异有统计学意义（Z=-19.915、16.831,P均<0.01）;临床症状方面,早发组强迫思维（χ²=11.998,P<0.05）、排序类（χ²=7.731,P<0.05）、仪式化类症状（χ²=7.714,P<0.05）比例高于晚发组,检查类（χ²=8.204,P<0.05）及清洗类（χ²=7.506,P<0.05）症状比例低于晚发组。共病神经发育障碍、共病情感障碍、有精神分裂症家族史、有情感障碍家族史是影响强迫症患者发病年龄的独立危险因素（OR=19.587、1.830、3.065、4.431,P均<0.05）,其中共病神经发育障碍是影响强迫症患者发病年龄的核心因素,女性是早发患者的保护因素（OR=0.417,P<0.01）。结论 早发和晚发型强迫症住院患者在人口学资料及临床症状上存在差异,共病神经发育障碍是影响强迫症住院患者起病年龄的核心危险因素。     

重症肌无力与胸腺外恶性肿瘤关系的临床研究

目的研究重症肌无力(MG)与胸腺外恶性肿瘤的相关性。方法研究2350例MG患者胸腺外恶性肿瘤的发生情况,并描述伴胸腺外恶性肿瘤MG患者的临床病理学特性。结果2350例MG患者中47例(2%)发现有胸腺外恶性肿瘤,最常见的肿瘤类型是肺癌。伴胸腺瘤MG患者,其胸腺外恶性肿瘤较非胸腺瘤患者显著增高。胸腺切除术、胸腺瘤分期及MG治疗方法不影响恶性肿瘤的发生。MG发病时Osserman分型,型者胸腺外恶性肿瘤较其它型少。结论MG患者胸腺外恶性肿瘤风险增加,尤其是伴胸腺瘤的MG患者,与胸腺切除、胸腺瘤分期及治疗方法无关。伴胸腺瘤性MG即使是良性的,也应随访注意有无其它恶性肿瘤的发生。     

Increasing incidence of late-onset anti-AChR antibody-seropositive myasthenia gravis

The incidence of myasthenia gravis (MG) from 1970 through 1999 was studied in an area with 2.3 million inhabitants. The mean annual incidence rate of early-onset MG was constant at 3.5 x 10(-6). In late-onset MG, the rate increased from 4.7 to 20.8 x 10(-6). The two onset types of MG may thus be distinct disorders. The author hypothesized that late-onset nonthymoma anti-acetylcholine receptor antibody-seropositive MG may be provoked by environmental factors.     

Thymectomy and anti-muscle autoantibodies in late-onset myasthenia gravis

Thymectomy is still widely carried out in myasthenia gravis (MG) patients, but its role, especially in late-onset MG patients, is not established. These patients are immunologically heterogeneous, some with thymoma-like and others with early onset-like features. We evaluated whether any therapeutic effects of thymectomy correlate with the presence of non-acetylcholine receptor (AChR) muscle antibodies. The severity of MG, and titin and ryanodine receptor (RyR) antibodies, were assessed yearly starting from MG onset in 21 thymectomized and 22 non-thymectomized AChR antibody positive late-onset MG patients, who were followed for 2, 3 and 5 years. Clinical or pharmacological remission were seen in six of 11 titin antibody negative but none of the 10 titin antibody positive thymectomized patients, however, the non-thymectomized cases showed an opposite trend. The three MG-related deaths were all in patients with titin antibodies. There was no significant difference in MG severity between thymectomized and non-thymectomized patients; 2 years after MG onset, both groups were significantly improved. This study showed no dramatic benefit from thymectomy in late-onset MG in general. Any limited improvement appeared less likely in cases with titin and/or RyR antibodies.