背景:Susac综合征(SuS)是一种罕见的自身免疫性疾病,可导致听力障碍,视野缺损,和脑内毛细血管前小动脉阻塞引起的脑病,视网膜,和内耳。鉴于潜在的灾难性结果以及区分SuS与鉴别诊断的困难,如多发性硬化症(MS),我们的探索性研究旨在鉴定潜在的新的SuS特异性神经影像学标志物.
方法:7例明确诊断为SuS的患者在7特斯拉(7T)时接受了磁共振成像(MRI),包括T2*加权和定量易感性作图(QSM)序列。T2加权高强度病变的数量进行了分析,volume,本地化,中央静脉征,T1低强度,和SuS病变中心的局灶性铁沉积物(“铁点”)。来自同一研究所的七个TMRI数据集,包括75名患者,其中,MS,作为控制。
结果:71.4%(5/7)的SuS患者存在“铁点”症状,与我们对照组的0%相比。因此,敏感性为71.4%,特异性为100%.仅偶然发现了中央静脉征象。
结论:我们是第一个在体内高分辨率7TT2*w和QSM图像上证明这种类型的“铁点”病变作为SuS的有前途的神经成像标志物,证实了以前的组织病理学离体发现。
Susac syndrome (SuS) is a rare autoimmune disease that leads to hearing impairment, visual field deficits, and encephalopathy due to an occlusion of precapillary arterioles in the brain, retina, and inner ear. Given the potentially disastrous outcome and difficulties in distinguishing SuS from its differential diagnoses, such as multiple sclerosis (MS), our exploratory study aimed at identifying potential new SuS-specific neuroimaging markers.
Seven patients with a definite diagnosis of SuS underwent magnetic resonance imaging (MRI) at 7 Tesla (7T), including T2* weighted and quantitative susceptibility mapping (QSM) sequences. T2 weighted hyperintense lesions were analyzed with regard to number, volume, localization, central vein sign, T1 hypointensity, and focal iron deposits in the center of SuS lesions (\"iron dots\"). Seven T MRI datasets from the same institute, comprising 75 patients with, among others, MS, served as controls.
The \"iron dot\" sign was present in 71.4% (5/7) of the SuS patients, compared to 0% in our control cohort. Thus, sensitivity was 71.4% and specificity 100%. A central vein sign was only incidentally detected.
We are the first to demonstrate this type of \"iron dot\" lesions on highly resolving 7T T2*w and QSM images in vivo as a promising neuroimaging marker of SuS, corroborating previous histopathological ex vivo findings.