Antulov Ronald, Holjar Erlic Izidora, Perkovic Olivio, Miletic Damir, Antoncic Igor
Department of Radiology, Clinical Hospital Center Rijeka, Rijeka, Croatia.
Department of Radiology, Clinical Hospital Center Rijeka, Rijeka, Croatia.
J Neurol Sci. 2014 Jun 15;341(1-2):162-4. doi: 10.1016/j.jns.2014.04.010. Epub 2014 Apr 13.
Susac's syndrome (SS) is an infrequent neurological disorder characterized by the clinical triad of encephalopathy, branch retinal artery occlusion and hearing loss due to an autoimmune endotheliopathy associated with anti-endothelial cell antibodies. At the onset of the disease SS rarely appears with the complete clinical triad. The most important diagnostic procedures involved in the diagnosis of SS are brain MRI, audiometric testing and retinal fluorescein angiography. Presence of at least two components of the SS clinical triad accompanied by specific brain MRI findings is highly suggestive of SS. We report a case of a young pregnant woman with a history of encephalopathy, hearing loss and walking impairment. Brain MRI revealed a spectrum of findings previously described in patients with SS. We induced labor at 37 weeks' gestation to start with immunosuppressive treatment and avoid possible fetal toxicity. To the best of our knowledge this is the first report of SS in Croatia.
苏萨克综合征（SS）是一种罕见的神经系统疾病，其特征为脑病、视网膜分支动脉阻塞和听力丧失这一临床三联征，病因是与抗内皮细胞抗体相关的自身免疫性内皮病变。在疾病发作时，SS很少以完整的临床三联征形式出现。SS诊断中最重要的诊断程序是脑部磁共振成像（MRI）、听力测试和视网膜荧光血管造影。SS临床三联征中至少有两个组成部分，并伴有特定的脑部MRI表现，高度提示为SS。我们报告一例有脑病、听力丧失和行走障碍病史的年轻孕妇病例。脑部MRI显示出先前在SS患者中描述过的一系列表现。我们在妊娠37周时引产，以便开始免疫抑制治疗并避免可能的胎儿毒性。据我们所知，这是克罗地亚首例SS报告。
