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巴林王国重症肌无力的临床描述。

Clinical delineation of myasthenia gravis in the Kingdom of Bahrain.

机构信息

From Department of Neurology (Binfalah), Department of Family and Community Medicine (Jaradat), Clinical Research Coordinator (Alhafnawi), Aljawhara Center (Bakhiet), Department of Molecular Medicine, Arabian Gulf University, Manama, and from the Neurology and Internal Medicine (Alhilly), Bahrain Defense Force Hospital, West Riffa, from Department of Internal Medicine (Al Nidawi, Alhammadi), King Hamad University Hospital, Busaiteen, from Department of Neurosciences (Abdulla), Salmanya Medical Complex, Ministry of Health, Juffair, Kingdom of Bahrain, and from the Department of Medicine (Shosha), Neurology Division, McMaster University, Hamilton, Canada.

出版信息

Neurosciences (Riyadh). 2022 Jan;27(1):16-23. doi: 10.17712/nsj.2022.1.20210096.

Abstract

OBJECTIVES

To report demographic and clinical data on 98 myasthenia gravis (MG) patients, seen over 5 years (January 2014-December 2018).

METHODS

This was a retrospective, observational cohort study carried out at 3 hospitals in Bahrain. MG was classified into ocular or generalized types. We subdivided MG into early-onset (EOMG, ≤ 49 years) or late-onset (LOMG, > 49 years). Demographic and clinical data were recorded. The data was entered and analyzed using SPSS version 26.0.

RESULTS

61.2% were females. The mean age at onset was 43.8±17.7 years in males and 43.1±15.7 years in females. 72.4% had EOMG. A pure ocular presentation was most common (51%). Limb weakness was more prevalent in AChR-positive patients. The MuSK group had more severe presentation. 57.1% of patients were AChR-positive, 3.1% MuSK-positive, and 39.8% double-seronegative. Generalized disease onset was more likely with AChR. Abnormal CT chest was seen in 24/69 (35%) including thymic hyperplasia, thymoma, and thymic atrophy. Pathology findings were thymic hyperplasia (55.0%), thymoma (30%), thymolipoma (10%), and normal thymus (5%). Treatment outcomes were favorable.

CONCLUSION

The present study revealed that MG was more common in females, with similar age at onset between males and females. The majority of patients had EOMG with ocular disease and AChR positivity. The clinical outcomes were favorable. Following a standardized protocol for MG diagnosis and workup is recommended.

摘要

目的

报告 98 例重症肌无力(MG)患者的人口统计学和临床数据,这些患者在 5 年内(2014 年 1 月至 2018 年 12 月)接受了治疗。

方法

这是一项在巴林 3 家医院进行的回顾性、观察性队列研究。MG 分为眼肌型或全身型。我们将 MG 分为早发型(EOMG,≤49 岁)或晚发型(LOMG,>49 岁)。记录了人口统计学和临床数据。数据使用 SPSS 版本 26.0 输入和分析。

结果

61.2%为女性。男性发病年龄的平均值为 43.8±17.7 岁,女性为 43.1±15.7 岁。72.4%为早发型。最常见的是单纯眼肌表现(51%)。AChR 阳性患者更易出现肢体无力。MuSK 组的表现更严重。57.1%的患者为 AChR 阳性,3.1%为 MuSK 阳性,39.8%为双阴性。AChR 更易导致全身型疾病发病。69 例中有 24 例(35%)胸部 CT 异常,包括胸腺增生、胸腺瘤和胸腺萎缩。病理发现为胸腺增生(55.0%)、胸腺瘤(30%)、胸腺脂肪瘤(10%)和正常胸腺(5%)。治疗效果良好。

结论

本研究表明,MG 在女性中更为常见,男女发病年龄相似。大多数患者为早发型,有眼肌疾病和 AChR 阳性。临床结局良好。建议按照 MG 诊断和检查的标准方案进行治疗。

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