胸腺切除术前疾病严重程度可预测乙酰胆碱受体抗体阳性全身性肌无力的预后。

IF 4.8 2区 医学 Q1 CLINICAL NEUROLOGY
Journal of Neurology Pub Date : 2024-09-01 Epub Date: 2024-07-30 DOI:10.1007/s00415-024-12592-x
Athanasios Papathanasiou, Chris R Tench, Philip A Ambrose, Saam Sedehizadeh, Radu Tanasescu
{"title":"胸腺切除术前疾病严重程度可预测乙酰胆碱受体抗体阳性全身性肌无力的预后。","authors":"Athanasios Papathanasiou, Chris R Tench, Philip A Ambrose, Saam Sedehizadeh, Radu Tanasescu","doi":"10.1007/s00415-024-12592-x","DOIUrl":null,"url":null,"abstract":"<p><strong>Introduction: </strong>There are only a few studies exploring post-thymectomy outcome in patients with acetylcholine receptor antibody (AChR-Ab)-positive generalised myasthenia gravis (MG).</p><p><strong>Objective: </strong>To assess the predictors of outcome in patients with AChR-Ab-positive generalised MG who underwent thymectomy.</p><p><strong>Methods: </strong>A retrospective study of 53 patients from a single neuroscience centre in the UK.</p><p><strong>Results: </strong>The mean disease duration from diagnosis was 6.2 ± 4.3 years. Pre-thymectomy, 37 patients had mild weakness affecting muscles other than ocular muscles, 11 patients had moderate weakness and 5 patients had severe weakness. 27/53 patients had thymoma. Post-thymectomy (mean duration of 5.7 ± 4.2 years), 34 patients (64%) had a good outcome characterised by Myasthenia Gravis Foundation of America Post-Intervention Status of complete stable remission (no symptoms or signs of MG for at least 1 year without any therapy) or pharmacological remission (no symptoms or signs of MG with some form of therapy) or minimal manifestations (no symptoms of functional limitations from MG but weakness on examination of some muscles with or without some form of therapy) on last follow-up visit. Having thymomatous or non-thymomatous MG did not predict the outcome. The only variable that did predict outcome was pre-thymectomy disease severity; patients with mild weakness before thymectomy had a favourable outcome. We found an accuracy of 83% predicting outcome (95% confidence interval (CI) 60%, 100%) with a sensitivity of 84% (95% CI 68%, 94%) and specificity of 81% (95% CI 54%, 96%).</p><p><strong>Conclusion: </strong>Disease severity before thymectomy predicts outcome in patients with AChR-Ab-positive generalised MG.</p>","PeriodicalId":16558,"journal":{"name":"Journal of Neurology","volume":null,"pages":null},"PeriodicalIF":4.8000,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"Pre-thymectomy disease severity predicts outcome in acetylcholine receptor antibody-positive generalised myasthenia gravis.\",\"authors\":\"Athanasios Papathanasiou, Chris R Tench, Philip A Ambrose, Saam Sedehizadeh, Radu Tanasescu\",\"doi\":\"10.1007/s00415-024-12592-x\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Introduction: </strong>There are only a few studies exploring post-thymectomy outcome in patients with acetylcholine receptor antibody (AChR-Ab)-positive generalised myasthenia gravis (MG).</p><p><strong>Objective: </strong>To assess the predictors of outcome in patients with AChR-Ab-positive generalised MG who underwent thymectomy.</p><p><strong>Methods: </strong>A retrospective study of 53 patients from a single neuroscience centre in the UK.</p><p><strong>Results: </strong>The mean disease duration from diagnosis was 6.2 ± 4.3 years. Pre-thymectomy, 37 patients had mild weakness affecting muscles other than ocular muscles, 11 patients had moderate weakness and 5 patients had severe weakness. 27/53 patients had thymoma. Post-thymectomy (mean duration of 5.7 ± 4.2 years), 34 patients (64%) had a good outcome characterised by Myasthenia Gravis Foundation of America Post-Intervention Status of complete stable remission (no symptoms or signs of MG for at least 1 year without any therapy) or pharmacological remission (no symptoms or signs of MG with some form of therapy) or minimal manifestations (no symptoms of functional limitations from MG but weakness on examination of some muscles with or without some form of therapy) on last follow-up visit. Having thymomatous or non-thymomatous MG did not predict the outcome. The only variable that did predict outcome was pre-thymectomy disease severity; patients with mild weakness before thymectomy had a favourable outcome. We found an accuracy of 83% predicting outcome (95% confidence interval (CI) 60%, 100%) with a sensitivity of 84% (95% CI 68%, 94%) and specificity of 81% (95% CI 54%, 96%).</p><p><strong>Conclusion: </strong>Disease severity before thymectomy predicts outcome in patients with AChR-Ab-positive generalised MG.</p>\",\"PeriodicalId\":16558,\"journal\":{\"name\":\"Journal of Neurology\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":4.8000,\"publicationDate\":\"2024-09-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Journal of Neurology\",\"FirstCategoryId\":\"3\",\"ListUrlMain\":\"https://doi.org/10.1007/s00415-024-12592-x\",\"RegionNum\":2,\"RegionCategory\":\"医学\",\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2024/7/30 0:00:00\",\"PubModel\":\"Epub\",\"JCR\":\"Q1\",\"JCRName\":\"CLINICAL NEUROLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of Neurology","FirstCategoryId":"3","ListUrlMain":"https://doi.org/10.1007/s00415-024-12592-x","RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/7/30 0:00:00","PubModel":"Epub","JCR":"Q1","JCRName":"CLINICAL NEUROLOGY","Score":null,"Total":0}
引用次数: 0

摘要

导言:只有少数研究探讨了乙酰胆碱受体抗体(AChR-Ab)阳性全身性肌无力(MG)患者胸腺切除术后的预后:评估接受胸腺切除术的乙酰胆碱受体抗体(AChR-Ab)阳性全身性肌无力患者的预后:方法:对英国一家神经科学中心的53名患者进行回顾性研究:结果:从诊断开始,平均病程为 6.2 ± 4.3 年。胸腺切除术前,37 名患者有轻度无力,影响到眼肌以外的肌肉,11 名患者有中度无力,5 名患者有重度无力。27/53 名患者患有胸腺瘤。胸腺切除术后(平均病程为 5.7 ± 4.2 年),34 名患者(64%)的疗效良好,其特征是在最后一次随访时,美国肌无力基金会的干预后状态为完全稳定缓解(在没有任何治疗的情况下,至少 1 年没有出现 MG 的症状或体征)或药物缓解(在接受某种治疗的情况下,没有出现 MG 的症状或体征)或表现轻微(在接受或不接受某种治疗的情况下,没有出现 MG 功能受限的症状,但在检查某些肌肉时会出现无力感)。胸腺瘤性或非胸腺瘤性 MG 无法预测结果。唯一能预测预后的变量是胸腺切除术前的疾病严重程度;胸腺切除术前轻度无力的患者预后较好。我们发现预测结果的准确率为 83%(95% 置信区间 (CI) 60%,100%),灵敏度为 84%(95% CI 68%,94%),特异性为 81%(95% CI 54%,96%):结论:胸腺切除术前的疾病严重程度可预测 AChR-Ab 阳性全身性 MG 患者的预后。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Pre-thymectomy disease severity predicts outcome in acetylcholine receptor antibody-positive generalised myasthenia gravis.

Introduction: There are only a few studies exploring post-thymectomy outcome in patients with acetylcholine receptor antibody (AChR-Ab)-positive generalised myasthenia gravis (MG).

Objective: To assess the predictors of outcome in patients with AChR-Ab-positive generalised MG who underwent thymectomy.

Methods: A retrospective study of 53 patients from a single neuroscience centre in the UK.

Results: The mean disease duration from diagnosis was 6.2 ± 4.3 years. Pre-thymectomy, 37 patients had mild weakness affecting muscles other than ocular muscles, 11 patients had moderate weakness and 5 patients had severe weakness. 27/53 patients had thymoma. Post-thymectomy (mean duration of 5.7 ± 4.2 years), 34 patients (64%) had a good outcome characterised by Myasthenia Gravis Foundation of America Post-Intervention Status of complete stable remission (no symptoms or signs of MG for at least 1 year without any therapy) or pharmacological remission (no symptoms or signs of MG with some form of therapy) or minimal manifestations (no symptoms of functional limitations from MG but weakness on examination of some muscles with or without some form of therapy) on last follow-up visit. Having thymomatous or non-thymomatous MG did not predict the outcome. The only variable that did predict outcome was pre-thymectomy disease severity; patients with mild weakness before thymectomy had a favourable outcome. We found an accuracy of 83% predicting outcome (95% confidence interval (CI) 60%, 100%) with a sensitivity of 84% (95% CI 68%, 94%) and specificity of 81% (95% CI 54%, 96%).

Conclusion: Disease severity before thymectomy predicts outcome in patients with AChR-Ab-positive generalised MG.

求助全文
通过发布文献求助,成功后即可免费获取论文全文。 去求助
来源期刊
Journal of Neurology
Journal of Neurology 医学-临床神经学
CiteScore
10.00
自引率
5.00%
发文量
558
审稿时长
1 months
期刊介绍: The Journal of Neurology is an international peer-reviewed journal which provides a source for publishing original communications and reviews on clinical neurology covering the whole field. In addition, Letters to the Editors serve as a forum for clinical cases and the exchange of ideas which highlight important new findings. A section on Neurological progress serves to summarise the major findings in certain fields of neurology. Commentaries on new developments in clinical neuroscience, which may be commissioned or submitted, are published as editorials. Every neurologist interested in the current diagnosis and treatment of neurological disorders needs access to the information contained in this valuable journal.
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
copy
已复制链接
快去分享给好友吧!
我知道了
右上角分享
点击右上角分享
0
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术官方微信