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在多发性硬化症诊所就诊以进行诊断的人群中顺磁性边缘病变的频率及诊断意义

Frequency and Diagnostic Implications of Paramagnetic Rim Lesions in People Presenting for Diagnosis to a Multiple Sclerosis Clinic.

作者信息

Renner Brian, Verter Elizabeth Daphne, Absinta Martina, Daboul Lynn, Raza Praneeta, Martin Melissa Lynne, Cao Quy, O'Donnell Carly M, Rodrigues Paulo R, Ramos Marc, Prchkovska Vesna, Derbyshire John, Azevedo Christina J, Bar-Or Amit, Caverzasi Eduardo, Calabresi Peter A, Cree Bruce Anthony Campbell, Freeman Leorah, Henry Roland G, Longbrake Erin E, Oh Jiwon, Papinutto Nico, Pelletier Daniel, Samudralwar Rohini D, Schindler Matthew K, Sotirchos Elias S, Sicotte Nancy L, Solomon Andrew J, Shinohara Russell T, Reich Daniel S, Ontaneda Daniel, Sati Pascal

机构信息

Department of Neurology, Cedars-Sinai Medical Center, Los Angeles, CA.

Department of Biomedical Sciences, Humanitas University, Milan, Italy.

出版信息

Neurology. 2025 Sep 23;105(6):e213912. doi: 10.1212/WNL.0000000000213912. Epub 2025 Sep 2.

DOI:10.1212/WNL.0000000000213912
PMID:40893055
Abstract

BACKGROUND AND OBJECTIVES

Paramagnetic rim lesions (PRLs) are a well-established imaging biomarker of chronic active multiple sclerosis (MS) lesions. PRLs have been shown to be highly specific for MS (∼90% specificity), and their prevalence has been estimated to be approximately 50% in patients with clinically established diagnoses of MS. In this study, we evaluated the frequency and diagnostic value of PRLs in patients at first clinical presentation.

METHODS

Adults age 18-64 years presenting with clinical symptoms or radiologic suspicion of demyelinating disease referred to academic specialty MS centers without a definitive diagnosis were prospectively enrolled in a multicenter, cross-sectional, observational study. Phase images from high-resolution 3D echo-planar imaging were acquired on 3-tesla brain MRI and evaluated for PRLs by 3 independent raters, blinded to diagnosis, with adjudication from a fourth expert rater. Diagnostic performance of PRLs for a diagnosis of MS using the 2017 McDonald criteria as gold standard was evaluated using diagnostic thresholds based on the presence of at least 1 PRL (≥1 PRL) or at least 2 PRLs (≥2 PRLs).

RESULTS

Seventy-eight participants were analyzed (mean age, years [range]: 45.0 [18-64] female sex n = 55 [71%]); a total of 124 PRLs were counted in 36 (46%) of the 78 participants (median: 3 PRLs; range: 1-9 PRLs). Thirty-two (89%) of the 36 PRL-positive participants fulfilled 2017 McDonald criteria, and the remaining 4 (11%) had an alternate diagnosis. The presence of ≥1 PRL had a sensitivity of 0.86 (95% CI 0.71-0.95) and a specificity of 0.90 (95% CI 0.77-0.97). For ≥2 PRLs, specificity increased to 0.95 (95% CI 0.83-0.99), whereas sensitivity decreased to 0.59 (95% CI 0.42-0.75). For participants with MS, shorter duration from initial symptom onset was associated with higher probability of having PRLs, with the odds of being PRL positive (≥1 PRL) increasing by 28% for every 1 year decrease from symptom onset (odds ratio 1.28 per year, 95% CI 1.03-1.59, = 0.03).

DISCUSSION

PRLs are highly prevalent early in patients with MS at the time of first clinical presentation and can differentiate MS from mimics with high accuracy.

摘要

背景与目的

顺磁性边缘病变(PRLs)是慢性活动性多发性硬化(MS)病变中一种公认的影像学生物标志物。PRLs已被证明对MS具有高度特异性(约90%的特异性),据估计,在临床确诊为MS的患者中,其患病率约为50%。在本研究中,我们评估了首次临床表现患者中PRLs的发生率及诊断价值。

方法

年龄在18 - 64岁、有临床症状或影像学怀疑为脱髓鞘疾病但未明确诊断的成年人,前瞻性纳入一项多中心、横断面、观察性研究,这些患者被转诊至学术专业MS中心。在3特斯拉脑部MRI上采集高分辨率3D回波平面成像的相位图像,并由3名独立的评估者在不知诊断结果的情况下对PRLs进行评估,最终由第四名专家评估者进行判定。以2017年麦克唐纳标准作为金标准,使用基于至少存在1个PRL(≥1个PRL)或至少2个PRLs(≥2个PRLs)的诊断阈值,评估PRLs对MS诊断的性能。

结果

共分析了78名参与者(平均年龄,岁[范围]:45.0[18 - 64];女性n = 55[71%]);在78名参与者中的36名(46%)中共计发现124个PRLs(中位数:3个PRLs;范围:1 - 9个PRLs)。36名PRL阳性参与者中有32名(89%)符合2017年麦克唐纳标准,其余4名(11%)有其他诊断。存在≥1个PRL时,敏感性为0.86(95%CI 0.71 - 0.95),特异性为0.90(95%CI 0.77 - 0.97)。对于≥2个PRLs,特异性增至0.95(95%CI 0.83 - 0.99),而敏感性降至0.59(95%CI 0.42 - 0.75)。对于患有MS的参与者,从初始症状发作起的病程越短,出现PRLs的可能性越高,从症状发作起每减少1年,PRL阳性(≥1个PRL)的几率增加28%(每年优势比1.28,95%CI 1.03 - 1.59,P = 0.03)。

讨论

PRLs在MS患者首次临床表现时早期发生率很高,并且能够高精度地区分MS与类似疾病。

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