Division of Molecular Neuroimmunology, Department of Neurology, University of Heidelberg, Im Neuenheimer Feld 400, D-69120 Heidelberg, Germany.
J Neurol Sci. 2009 Oct 15;285(1-2):259-61. doi: 10.1016/j.jns.2009.07.002. Epub 2009 Jul 29.
Susac's syndrome (SS) is a rare arteriopathy affecting the microvasculature of the brain, retina, and inner ear, resulting in encephalopathy, branch retinal artery occlusion and hearing loss. Anecdotal reports exist on SS being associated with a humoral immune response against endothelial cells. However, no original data has ever been published.
To analyze serum and CSF from a patient with SS for the presence of CNS auto-antibodies and, if present, to further characterize such antibodies immunologically.
Serum and CSF samples were examined by indirect immunofluorescence on adult mouse cerebrum, cerebellum, brain stem, and inner ear tissue sections, and IgG subclasses were determined.
Anti-endothelial antibodies were found at a titre of 1:960 in serum but not CSF. Antibodies belonged to the complement activating IgG1 subclass. Glucocorticoid treatment resulted in a decrease of titres (1:480), though the antibodies remained clearly detectable.
Our finding of anti-endothelial cell antibodies in a patient with SS is important in the light of previous pathological data suggesting that SS is associated with endothelial damage. Larger serological studies are now warranted to assess systematically the frequency and relevance of auto-antibodies in SS.
苏萨克斯综合征（SS）是一种罕见的小动脉病，影响大脑、视网膜和内耳的微血管，导致脑病、视网膜分支动脉阻塞和听力损失。有一些关于 SS 与针对内皮细胞的体液免疫反应有关的传闻报告。然而，从未发表过原始数据。
分析 SS 患者的血清和脑脊液中是否存在中枢神经系统自身抗体，如果存在，进一步从免疫学角度对这些抗体进行特征分析。
通过间接免疫荧光法在成年小鼠大脑、小脑、脑干和内耳组织切片上检测血清和脑脊液样本，并确定 IgG 亚类。
血清中抗内皮细胞抗体的滴度为 1:960，但脑脊液中未检测到。抗体属于补体激活 IgG1 亚类。糖皮质激素治疗后滴度降低（1:480），但抗体仍明显可检测到。
我们在 SS 患者中发现抗内皮细胞抗体，这与先前的病理学数据表明 SS 与内皮损伤有关，这一点很重要。现在需要进行更大的血清学研究，以系统评估自身抗体在 SS 中的频率和相关性。
