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极晚发型全身型重症肌无力的短期预后:一项单中心回顾性队列研究

The short-term prognosis of very late onset generalized myasthenia gravis: a single-center retrospective cohort study.

作者信息

Liampas Ioannis, Veltsista Dimitra, Batzikosta Paraskevi, Lazarou Lefteris, Kefalopoulou Zinovia Maria, Chroni Elisabeth

机构信息

Neuromuscular Center, Department of Neurology, University Hospital of Patras, Patras, Greece.

Department of Neurology, University Hospital of Larissa, School of Medicine, University of Thessaly, Larissa, Greece.

出版信息

Front Neurol. 2025 Aug 11;16:1641701. doi: 10.3389/fneur.2025.1641701. eCollection 2025.

DOI:10.3389/fneur.2025.1641701
PMID:40860974
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12375450/
Abstract

OBJECTIVE

To explore the short-term prognosis of generalized very-late-onset myasthenia gravis (vloMG, symptom onset ≥65 years) in comparison with early- and late-onset MG (eloMG, <65 years).

METHODS

A single-center retrospective cohort study was conducted based on the medical records of patients with laboratory confirmed generalized MG, monitored in the specialized Unit of Neuromuscular Disorders of the University Hospital of Patras. Measures of clinical severity were compared at baseline and over the short term (2-year) follow-up.

RESULTS

There were 42 eligible patients (42.1 ± 13.2 years, 50% women, 19.5 ± 6.0 months follow-up) in the eloMG and 26 (72.4 ± 5.0, 50% women, 13.9 ± 7.9 months follow-up) in the vloMG group. In the vloMG group, AchR antibody positivity (89% vs. 57%,  = 0.007) and oculo-bulbar symptoms at onset (88% vs. 53%,  = 0.002) were more common, whereas thymus pathology (0% vs. 40%,  < 0.001) and generalized weakness at onset (12% vs. 38%,  = 0.018) were less frequent. Intubation within the first month from diagnosis was required only in patients with vloMG (5/26) ( = 0.006). Over the follow-up: the unadjusted incidence rate ratio (IRR) of relapses was lower in the vloMG group [IRR = 0.49, 95% CI = (0.26, 0.92),  = 0.026], the unadjusted odds (OR) of being classified as <IIb on the MGFA classification were higher in the vloMG group [OR = 2.27 95% CI = (1.02, 5.05),  = 0.043] and the average unadjusted difference in corticosteroid intake was lower in the vloMG group by approximately 6.9 mg [(-10.6, 3.3),  < 0.001] in equivalent doses of prednisolone [6.1 mg (-10.0, -2.2),  = 0.002, according to adjusted estimations].

CONCLUSION

Despite its more aggressive onset, vloMG has a more favorable prognosis than eloMG.

摘要

目的

探讨晚发型重症肌无力(vloMG,症状出现≥65岁)与早发型和晚发型重症肌无力(eloMG,<65岁)相比的短期预后。

方法

基于在帕特雷大学医院神经肌肉疾病专科监测的实验室确诊的全身型重症肌无力患者的病历进行单中心回顾性队列研究。在基线和短期(2年)随访期间比较临床严重程度指标。

结果

eloMG组有42例符合条件的患者(42.1±13.2岁,50%为女性,随访19.5±6.0个月),vloMG组有26例(年龄72.4±5.0岁,50%为女性,随访13.9±7.9个月)。在vloMG组中,乙酰胆碱受体抗体阳性(89%对57%,P = 0.007)和发病时的眼咽症状(88%对53%,P = 0.002)更为常见,而胸腺病理改变(0%对40%,P<0.001)和发病时的全身无力(12%对38%,P = 0.018)则较少见。仅vloMG组的患者(5/26)在诊断后的第一个月内需要插管(P = 0.006)。在随访期间:vloMG组复发的未调整发病率比(IRR)较低[IRR = 0.49,95%置信区间=(0.26,0.92),P = 0.026],vloMG组在MGFA分类中被归类为<IIb的未调整比值比(OR)较高[OR = 2.27,95%置信区间=(1.02,5.05),P = 0.043],且vloMG组皮质类固醇摄入量的平均未调整差异以泼尼松龙等效剂量计算约低6.9 mg[(-10.6,3.3),P<0.001][根据调整后的估计,为6.1 mg(-10.0,-2.2),P = 0.002]。

结论

尽管vloMG起病更急,但与eloMG相比预后更有利。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9954/12375450/4d3804014e03/fneur-16-1641701-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9954/12375450/3f9567dfbb46/fneur-16-1641701-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9954/12375450/4d3804014e03/fneur-16-1641701-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9954/12375450/3f9567dfbb46/fneur-16-1641701-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9954/12375450/4d3804014e03/fneur-16-1641701-g002.jpg

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