Clinical Characteristics and Outcomes of Generalized Myasthenia Gravis in Malaysia: A Single-Center Experience.

IF 2.9 3区 医学 Q2 CLINICAL NEUROLOGY
Jie Ying Tan, Cheng Yin Tan, Prasana Nair Gengadharan, Nortina Shahrizaila, Khean Jin Goh
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引用次数: 0

Abstract

Background and purpose: Myasthenia gravis (MG) is clinically heterogeneous and can be classified into subgroups according to the clinical presentation, antibody status, age at onset, and thymic abnormalities. This study aimed to determine the clinical characteristics and outcomes of generalized MG (GMG) patients based on these subgroups.

Methods: Medical records of MG patients from 1976 to 2023 were reviewed retrospectively. Patients with pure ocular MG were excluded. Data on demographic, clinical characteristics, laboratory features, and outcomes were analyzed.

Results: This study included 120 GMG patients. There was a slight preponderance of female patients over male patients (male:female ratio=1:1.3), with the age at onset exhibiting a bimodal distribution. Female patients peaked at a lower age (21-30 years) whereas male patients peaked at a higher age (61-70 years). Most (92%, 105 of 114) patients had positive anti-acetylcholine receptor antibodies. Five patients were also tested for anti-muscle-specific tyrosine kinase antibodies, with two showing positivity. Thymectomy was performed in 62 (52%) patients, of which 30 had thymoma, 16 had thymic hyperplasia, 7 had an involuted thymus, and 6 had a normal thymus. There were significantly more female patients (68% vs. 45%, p=0.011) with early-onset disease (<50 years old) and thymic hyperplasia (33% vs. 0%, p<0.025). Most (71%) of the patients had a good outcome based on the Myasthenia Gravis Foundation of America postintervention status. GMG patients with early-onset disease had a significantly better outcome than patients with a late onset in univariate (58% vs. 37%, p=0.041) and multivariate (odds ratio=4.68, 95% confidence interval=1.17-18.64, p=0.029) analyses.

Conclusions: Female patients with early-onset MG and thymic hyperplasia had significantly better outcomes, but only early-onset disease was independently associated with a good outcome. These findings are comparable with those of other studies.

马来西亚全身性肌无力 Gravis 的临床特征和疗效:单中心经验
背景和目的:重症肌无力(MG)在临床上具有异质性,可根据临床表现、抗体状态、发病年龄和胸腺异常分为不同的亚组。本研究旨在根据这些亚组确定全身型肌萎缩症(GMG)患者的临床特征和预后:方法:回顾性分析了1976年至2023年间MG患者的医疗记录。方法:对 1976 年至 2023 年的 MG 患者病历进行了回顾性研究,排除了纯眼部 MG 患者。分析了人口统计学、临床特征、实验室特征和结果等数据:本研究共纳入 120 名 GMG 患者。女性患者略多于男性患者(男女比例=1:1.3),发病年龄呈双峰分布。女性患者的发病高峰年龄较低(21-30 岁),而男性患者的发病高峰年龄较高(61-70 岁)。大多数患者(92%,114 人中的 105 人)的抗乙酰胆碱受体抗体呈阳性。五名患者还接受了抗肌肉特异性酪氨酸激酶抗体检测,其中两人呈阳性。62名(52%)患者接受了胸腺切除术,其中30人患有胸腺瘤,16人患有胸腺增生,7人胸腺内陷,6人胸腺正常。在早期发病(pp=0.041)和多变量(几率比=4.68,95% 置信区间=1.17-18.64,p=0.029)分析中,女性患者明显较多(68% 对 45%,p=0.011):结论:早发MG和胸腺增生的女性患者的预后明显更好,但只有早发疾病才与良好预后独立相关。这些研究结果与其他研究结果相似。
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来源期刊
Journal of Clinical Neurology
Journal of Clinical Neurology 医学-临床神经学
CiteScore
4.50
自引率
6.50%
发文量
0
审稿时长
>12 weeks
期刊介绍: The JCN aims to publish the cutting-edge research from around the world. The JCN covers clinical and translational research for physicians and researchers in the field of neurology. Encompassing the entire neurological diseases, our main focus is on the common disorders including stroke, epilepsy, Parkinson''s disease, dementia, multiple sclerosis, headache, and peripheral neuropathy. Any authors affiliated with an accredited biomedical institution may submit manuscripts of original articles, review articles, and letters to the editor. The JCN will allow clinical neurologists to enrich their knowledge of patient management, education, and clinical or experimental research, and hence their professionalism.
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