Johns Hopkins University, School of Medicine, Department of Neurology, Baltimore, MD, USA; Johns Hopkins University School of Nursing, Baltimore, MD, USA.
Johns Hopkins University School of Nursing, Baltimore, MD, USA.
Mult Scler Relat Disord. 2019 Feb;28:64-68. doi: 10.1016/j.msard.2018.12.011. Epub 2018 Dec 9.
Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune disease of the central nervous system (CNS) that preferentially targets the spinal cord and optic nerves. Increasing disability is accrued with each inflammatory attack. Disability has been shown to be an independent predictor of poor quality of life in those with NMOSD. Factors associated with increasing disability need further systematic investigation.
We performed a multi-center retrospective chart analysis of aquaporin-4 (AQP4) seropositive NMOSD patients with a history of myelitis seen at five large referral centers for patients with NMOSD worldwide for whom thorough records including relapse history and corresponding imaging were available. Potential contributors to long-term disability were extracted including demographics, radiographic findings, and clinical characteristics. Multivariable regression modeling was conducted to determine correlates of disability in patients with NMOSD, as measured by the Expanded Disability Status Scale (EDSS).
One hundred eighty-two AQP4 seropositive patients (88% female) were included in this analysis. Multiple regression modeling revealed that older age at disease onset, delay in diagnosis/preventive treatment, length of longest acute myelitis lesion and presence of symptomatic brain/brainstem lesions were associated with increased disability when holding other variables constant.
While age at onset is a factor that cannot be controlled in NMOSD, we can reduce the delay in diagnosis/preventive treatment and reduce future relapses in the brain/brainstem and spinal cord. Delay in diagnosis/preventive treatment and imaging variables that contributed to increased disability support the need for improved measures for early, accurate diagnosis and management of NMOSD, and aggressive treatment of acute relapses.
视神经脊髓炎谱系疾病（NMOSD）是一种中枢神经系统（CNS）自身免疫性疾病，优先靶向脊髓和视神经。每次炎症发作都会增加残疾程度。残疾已被证明是 NMOSD 患者生活质量差的独立预测因素。需要进一步系统研究与残疾程度增加相关的因素。
我们对五家大型 NMOSD 转诊中心的 NMOSD 患者进行了回顾性图表分析，这些患者的 AQP4 血清阳性，且有脊髓炎病史，全球有详细记录包括复发史和相应影像学资料。提取与长期残疾相关的潜在因素，包括人口统计学、影像学表现和临床特征。采用多变量回归模型确定 NMOSD 患者残疾的相关因素，用扩展残疾状况量表（EDSS）来衡量。
本研究共纳入 182 例 AQP4 血清阳性患者（88%为女性）。多变量回归模型显示，发病年龄较大、诊断/预防治疗延迟、最长急性脊髓炎病灶长度和有症状性脑/脑干病灶与残疾程度增加有关，在其他变量不变的情况下。
虽然发病年龄是 NMOSD 无法控制的因素，但我们可以减少诊断/预防治疗的延迟，并减少未来脑/脑干和脊髓的复发。诊断/预防治疗延迟和影像学变量与残疾程度增加有关，这支持需要改进 NMOSD 的早期、准确诊断和管理措施，并积极治疗急性复发。
