光谱域光学相干断层扫描检测到的 Susac 综合征的视网膜病变。
Retinal pathology in Susac syndrome detected by spectral-domain optical coherence tomography.
机构信息
From the Departments of Neurology (M.R., P.A., B.B., J.H., A.-K.M., H.-P.H., O.A.) and Ophthalmology (D.F., R.G.), Medical Faculty, Heinrich-Heine University Düsseldorf; the Department of Neurology (I.K., T.D.), University of Münster; the Department of Ophthalmology (R.B.), NeuroCure Clinical Research Center (F.P., A.B., T.O., J.M., J.D.), and Clinical and Experimental Multiple Sclerosis Research Center, Department of Neurology (F.P., J.D.), Charité-Universitätsmedizin Berlin; the Department of Neurology (M.K.), Alfried Krupp Hospital, Essen; and Molecular Neuroimmunology (B.W., S.J.), Department of Neurology, University of Heidelberg, Germany.
出版信息
Neurology. 2015 Aug 18;85(7):610-8. doi: 10.1212/WNL.0000000000001852. Epub 2015 Jul 22.
OBJECTIVE
The aim of this non-interventional study was to characterize retinal layer pathology in Susac syndrome (SuS), a disease with presumably autoimmune-mediated microvessel occlusions in the retina, brain, and inner ear, in comparison to the most important differential diagnosis multiple sclerosis (MS).
METHODS
Seventeen patients with SuS and 17 age- and sex-matched patients with relapsing-remitting MS (RRMS) and healthy controls (HC) were prospectively investigated by spectral-domain optical coherence tomography (OCT) including intraretinal layer segmentation in a multicenter study. Patients with SuS additionally received retinal fluorescein angiography (FA) and automated perimetry.
RESULTS
Patchy thinning of the retinal nerve fiber layer, ganglion cell layer, inner plexiform layer, inner nuclear layer, and outer plexiform layer compared to corresponding sectors in RRMS and HC eyes (p < 0.003 for SuS vs RRMS and HC) was observed in 23/34 (68%) SuS eyes, particularly in temporal quadrants. The outer nuclear layer (ONL) and photoreceptor layers (PRL) were not affected. FA performed in 15/17 patients with SuS was negative for disease-specific branch retinal artery occlusions in all but 1 eye at the time of OCT examination and revealed no additional vascular abnormalities, even in severely damaged OCT areas. In a subset of patients with SuS, associations of visual field data with distinct retinal layers were observed.
CONCLUSION
Distinct OCT patterns of scattered, scar-like intraretinal pathology in SuS eyes, sparing the ONL and PRL, suggest a retinal, but not choroidal, vascular pathomechanism and clearly differentiate SuS from RRMS. Depending on the disease stage, OCT and FA provide specific complementary diagnostic information in SuS.
目的
本非介入性研究旨在对 Susac 综合征（SuS）的视网膜层病理进行特征描述，该疾病被认为是视网膜、大脑和内耳中小血管自身免疫介导的闭塞，与最重要的鉴别诊断多发性硬化症（MS）进行比较。
方法
在一项多中心研究中，我们前瞻性地检查了 17 例 SuS 患者、17 例年龄和性别匹配的复发缓解型多发性硬化症（RRMS）患者和健康对照者（HC），采用频域光相干断层扫描（OCT），包括视网膜内部分层。SuS 患者还接受了视网膜荧光素血管造影（FA）和自动视野检查。
结果
与 RRMS 和 HC 眼相应部位相比，34 只 SuS 眼中有 23 只（68%）出现视网膜神经纤维层、节细胞层、内丛状层、内核层和外丛状层的斑片状变薄（p < 0.003 为 SuS 与 RRMS 和 HC），尤其是在颞象限。外核层（ONL）和光感受器层（PRL）不受影响。在 15/17 例接受 SuS 的患者中进行的 FA 在 OCT 检查时除 1 只眼外均为阴性，无疾病特异性分支视网膜动脉闭塞，甚至在 OCT 严重受损区域也未发现其他血管异常。在 SuS 患者的亚组中，观察到视野数据与特定视网膜层之间存在关联。
结论
SuS 眼中散在的、瘢痕样的视网膜内病理 OCT 模式，保留了 ONL 和 PRL，提示视网膜而非脉络膜的血管发病机制，可将 SuS 与 RRMS 明确区分开来。根据疾病阶段，OCT 和 FA 在 SuS 中提供特定的互补诊断信息。
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