Clinica Neurologica, Dipartimento di Medicina e Chirurgia, Università degli Studi di Perugia, Perugia, Italy.
Azienda Ospedaliero-Universitaria "San Giovanni di Dio e Ruggi d'Aragona", Salerno, Italy.
Eur J Neurol. 2021 Apr;28(4):1299-1307. doi: 10.1111/ene.14672. Epub 2021 Jan 12.
Tumefactive multiple sclerosis (TuMS) (i.e., MS onset presenting with tumefactive demyelinating lesions [TDLs]) is a diagnostic and therapeutic challenge. We performed a multicentre retrospective study to describe the clinical characteristics and the prognostic factors of TuMS.
One hundred two TuMS patients were included in this retrospective study. Demographic, clinical, magnetic resonance imaging (MRI), laboratory data and treatment choices were collected.
TuMS was found to affect women more than men (female:male: 2.4), with a young adulthood onset (median age: 29.5 years, range: 11-68 years, interquartile range [IQR]: 38 years). At onset, 52% of TuMS patients presented with the involvement of more than one functional system and 24.5% of them with multiple TDLs. TDLs most frequently presented with an infiltrative MRI pattern (38.7%). Cerebrospinal fluid immunoglobulin G oligoclonal bands were often demonstrated (76.6%). In 25.3% of the cases, more than one acute-phase treatment was administered, and almost one-half of the patients (46.6%) were treated with high-efficacy treatments. After a median follow-up of 2.3 years (range: 0.1-10.7 years, IQR: 3.4 years), the median Expanded Disability Status Scale (EDSS) score was 1.5 (range: 0-7, IQR: 2). Independent risk factors for reaching an EDSS score ≥3 were a higher age at onset (odds ratio [OR]: 1.08, 95% confidence interval [CI]: 1.03-1.14, p < 0.01), a higher number of TDLs (OR: 1.67, 95% CI: 1.02-2.74, p < 0.05) and the presence of infiltrative TDLs (OR: 3.34, 95% CI: 1.18-9.5, p < 0.001) at baseline.
The management of TuMS might be challenging because of its peculiar characteristics. Large prospective studies could help to define the clinical characteristics and the best treatment algorithms for people with TuMS.
肿块性多发性硬化症(TuMS)(即多发性硬化症以肿块性脱髓鞘病变[TDL]起病)是一种诊断和治疗上的挑战。我们进行了一项多中心回顾性研究,以描述 TuMS 的临床特征和预后因素。
本回顾性研究纳入了 102 例 TuMS 患者。收集了人口统计学、临床、磁共振成像(MRI)、实验室数据和治疗选择。
TuMS 更常见于女性(女性:男性=2.4),且在成年早期发病(中位年龄:29.5 岁,范围:11-68 岁,四分位距[IQR]:38 岁)。起病时,52%的 TuMS 患者有超过一个功能系统受累,24.5%的患者有多个 TDL。TDL 最常表现为浸润性 MRI 模式(38.7%)。脑脊液免疫球蛋白 G 寡克隆带常呈阳性(76.6%)。25.3%的病例接受了不止一种急性期治疗,近一半的患者(46.6%)接受了高效治疗。中位随访 2.3 年后(范围:0.1-10.7 年,IQR:3.4 年),扩展残疾状态量表(EDSS)的中位数评分为 1.5(范围:0-7,IQR:2)。达到 EDSS 评分≥3 的独立危险因素是发病年龄较高(优势比[OR]:1.08,95%置信区间[CI]:1.03-1.14,p<0.01)、TDL 数量较多(OR:1.67,95%CI:1.02-2.74,p<0.05)和基线时存在浸润性 TDL(OR:3.34,95%CI:1.18-9.5,p<0.001)。
由于其特殊特征,TuMS 的治疗可能具有挑战性。大型前瞻性研究有助于确定 TuMS 患者的临床特征和最佳治疗方案。