Neuroimaging Research Unit, Division of Neuroscience, IRCCS San Raffaele Scientific Institute, Milan, Italy.
Neurology Unit, IRCCS San Raffaele Scientific Institute, Milan, Italy.
Ann Neurol. 2022 Aug;92(2):173-183. doi: 10.1002/ana.26419. Epub 2022 Jun 11.
Astrocytes outline the perivascular space (PVS) and regulate fluid exchange through the aquaporin-4 water channel. As neuromyelitis optica is an autoimmune astrocytopathy targeting aquaporin-4, we hypothesized that it could be associatied with PVS abnormalities.
A total of 34 patients, and 46 age- and sex-matched healthy controls from two independent cohorts (exploratory and validation dataset) underwent a standardized 3.0-T magnetic resonance imaging protocol including conventional and diffusion tensor imaging. Susceptibility-weighted imaging was also acquired in the exploratory dataset. We evaluated macroscopic and microstructural abnormalities of PVS in terms of enlargement and water diffusivity (DTI-ALPS index). In the exploration dataset, a susceptibility-weighted sequence was used to draw the regions of interest for the DTI-ALPS index calculation in areas having veins perpendicular to lateral ventricles. Between-group comparisons, correlations, and regression models were run to assess associations between PVS abnormalities, and clinical and magnetic resonance imaging variables.
Patients had a higher frequency of severe PVS enlargement in the centrum semiovale (29.4% vs 8.7%), which correlated with brain atrophy, deep grey matter atrophy, and poorer cognitive performance (r-values range: -0.44, -0.36; p values: 0.01-0.046). In both datasets, patients had reduced DTI-ALPS index compared with controls (p values 0.004-0.038). Lower DTI-ALPS index, deep gray matter volume, and cortical volume could discriminate between patients and controls (R = 0.62), whereas lower DTI-ALPS index, higher number of myelitis, and higher T2-lesion volume were associated with worse disability (R = 0.55).
Patients with neuromyelitis optica spectrum disorder are characterized by abnormal enlargement and impaired water diffusion along the PVS, whose clinical implications suggest a direct correlation with disease pathogenesis and severity. ANN NEUROL 2022;92:173-183.
星形胶质细胞围绕着血管周围间隙(PVS),并通过水通道蛋白-4 调节液体交换。由于视神经脊髓炎是一种针对水通道蛋白-4 的自身免疫性星形胶质细胞病,我们假设它可能与 PVS 异常有关。
共纳入来自两个独立队列(探索性和验证性数据集)的 34 名患者和 46 名年龄和性别匹配的健康对照者,他们接受了包括常规和弥散张量成像在内的标准化 3.0T 磁共振成像方案。在探索性数据集还采集了磁共振磁敏感加权成像。我们根据 PVS 扩大和水扩散率(DTI-ALPS 指数)评估 PVS 的宏观和微观结构异常。在探索性数据集,使用磁敏感加权序列在与侧脑室垂直的静脉所在区域绘制 DTI-ALPS 指数计算的感兴趣区。进行组间比较、相关性和回归模型,以评估 PVS 异常与临床和磁共振成像变量之间的关系。
患者在半卵圆中心的 PVS 扩大更为严重(29.4%比 8.7%),与脑萎缩、深部灰质萎缩和认知功能下降相关(r 值范围:-0.44 至-0.36;p 值:0.01 至 0.046)。在两个数据集,患者的 DTI-ALPS 指数均低于对照组(p 值分别为 0.004 至 0.038)。较低的 DTI-ALPS 指数、深部灰质体积和皮质体积可以区分患者和对照组(R=0.62),而较低的 DTI-ALPS 指数、多发性脊髓炎和更高的 T2 病变体积与更差的残疾相关(R=0.55)。
视神经脊髓炎谱系障碍患者的特点是 PVS 沿异常扩大和水扩散受损,其临床意义表明与疾病发病机制和严重程度直接相关。ANN NEUROL 2022;92:173-183.