PDF(Pubmed)
Abstract：
We present a 20-year-old woman who was diagnosed with subacute sclerosing panencephalitis (SSPE) 20 months after presenting with unilateral retinitis. At presentation, the patient had two inferotemporal macular lesions in her left eye. Corresponding to these areas, optical coherence tomography (OCT) showed hyporeflective spaces with loss of nearly all of the retinal layers. OCT-angiography (OCTA) demonstrated some flow deficit areas with a reduction in the vessel density. Her serum measles antibody titre was high (IgG >5000.0 mIU/ml). Twenty months later the macular lesions had diminished in size, and there was some focal retinal thinning with interruption of the ellipsoid zone. OCTA showed that the flow deficit areas were diminished in size together with the relatively improved perfusion density. Neurological examination disclosed myoclonic jerks. Neuropsychological assessment demonstrated impaired executive function, attention, and narrowed lexical fluency. Measles IgG antibody was high in the cerebrospinal fluid (>230.0 U/ml). Brain magnetic resonance imaging demonstrated bilateral, non-specific, small foci of T2 hyperintensity in the frontoparietal subcortical white matter and centrum semiovale. The present case is the first where OCTA findings of SSPE-related retinal lesions have been described.
摘要：
我们介绍了一名20岁的女性，她在出现单侧视网膜炎20个月后被诊断为亚急性硬化性全脑炎（SSPE）。在介绍时,该患者的左眼有两个下颞叶黄斑病变。对应于这些地区,光学相干断层扫描(OCT)显示低反射空间,几乎所有的视网膜层都消失.OCT血管造影术（OCTA）显示一些流量不足区域，血管密度降低。她的血清麻疹抗体滴度高（IgG>5000.0mIU/ml）。20个月后，黄斑病变缩小了，有一些局灶性视网膜变薄，椭圆形区中断。OCTA显示，流量不足区域的大小随着灌注密度的相对提高而减小。神经系统检查显示肌阵挛性抽搐。神经心理学评估显示执行功能受损，注意,和缩小词汇流畅性。脑脊液中麻疹IgG抗体高（>230.0U/ml）。脑磁共振成像显示双侧，非特异性,额顶叶皮质下白质和中心半卵中T2高强度的小病灶。本病例是首次描述SSPE相关视网膜病变的OCTA发现。
