Senior Department of Neurology, The First Medical Center of People's Liberation Army (PLA) General Hospital, Beijing, China.
Department of Neurosurgery, Xuanwu Hospital, Capital Medical University, Beijing, China.
Front Immunol. 2022 Dec 1;13:1052678. doi: 10.3389/fimmu.2022.1052678. eCollection 2022.
To track the clinical outcomes in patients who initially presented with tumefactive demyelinating lesions (TDLs), we summarized the clinical characteristics of various etiologies, and identified possible relapse risk factors for TDLs.
Between 2001 and 2021, 116 patients initially presented with TDLs in our hospital were retrospectively evaluated. Patients were followed for relapse and clinical outcomes, and grouped according to various etiologies. Demographic information, clinical data, imaging data, and laboratory results of patients were obtained and analyzed. The risk factors of relapse were analyzed by the Log-Rank test and the Cox proportional hazard model in multivariate analysis.
During a median follow-up period of 72 months, 33 patients were diagnosed with multiple sclerosis (MS), 6 patients with Balo, 6 patients with neuromyelitis optica spectrum disorders (NMOSD), 10 patients with myelin oligodendrocyte glycoprotein antibody-associated demyelination (MOGAD), 1 patient with acute disseminated encephalomyelitis (ADEM), and the remaining 60 patients still have no clear etiology. These individuals with an unknown etiology were categorized independently and placed to the other etiology group. In the other etiology group, 13 patients had recurrent demyelinating phases, while 47 patients did not suffer any more clinical events. Approximately 46.6% of TDLs had relapses which were associated with multiple functional system involvement, first-phase Expanded Disability Status Scale score, lesions morphology, number of lesions, and lesions location (0.05). And diffuse infiltrative lesions (=0.003, =6.045, 95%:1.860-19.652), multiple lesions (=0.001, =3.262, 95%:1.654-6.435) and infratentorial involvement (=0.006, =2.289, 95%:1.064-3.853) may be independent risk factors for recurrence. Relapse free survival was assessed to be 36 months.
In clinical practice, around 46.6% of TDLs relapsed, with the MS group showing the highest recurrence rate, and lesions location, diffuse infiltrative lesions, and multiple lesions might be independent risk factors for relapse. Nevertheless, despite extensive diagnostic work and long-term follow-up, the etiology of TDLs in some patients was still unclear. And these patients tend to have monophase course and a low rate of relapse.
为了跟踪最初表现为肿块样脱髓鞘病变(TDL)患者的临床转归,我们总结了不同病因的临床特征,并确定了 TDL 可能的复发风险因素。
回顾性分析 2001 年至 2021 年期间在我院就诊的 116 例最初表现为 TDL 的患者。对患者进行复发和临床结局随访,并根据不同病因进行分组。获取并分析患者的人口统计学信息、临床资料、影像学资料和实验室结果。通过对数秩检验和 Cox 比例风险模型对多因素分析中的复发危险因素进行分析。
在中位随访 72 个月期间,33 例患者被诊断为多发性硬化(MS),6 例为 Balo 病,6 例为视神经脊髓炎谱系疾病(NMOSD),10 例为髓鞘少突胶质细胞糖蛋白抗体相关脱髓鞘(MOGAD),1 例为急性播散性脑脊髓炎(ADEM),其余 60 例患者仍病因不明。这些病因不明的患者被单独归类为其他病因组。在其他病因组中,13 例患者出现复发性脱髓鞘期,而 47 例患者未发生任何其他临床事件。约 46.6%的 TDL 发生了复发,复发与多系统受累、首发期扩展残疾状况量表(EDSS)评分、病变形态、病变数量和病变位置相关(0.05)。弥漫浸润性病变(=0.003,=6.045,95%CI:1.860-19.652)、多发病变(=0.001,=3.262,95%CI:1.654-6.435)和幕下累及(=0.006,=2.289,95%CI:1.064-3.853)可能是复发的独立危险因素。无复发生存率评估为 36 个月。
在临床实践中,约 46.6%的 TDL 会复发,其中 MS 组复发率最高,病变位置、弥漫浸润性病变和多发病变可能是复发的独立危险因素。然而,尽管进行了广泛的诊断工作和长期随访,仍有部分患者的 TDL 病因仍不明确。这些患者往往表现为单相病程,复发率较低。