Søgaard Lisa Eilenberg, Illes Zsolt, Nguyen Nina, Byg Keld-Erik
Ugeskr Laeger. 2019 Apr 8;181(15).
This is a case report of a 56-year-old male patient with Susac syndrome. The syndrome is a rare immune-mediated, ischaemic, occlusive microvascular endotheliopathy affecting the brain, retina and inner ear. Diagnosis and treatment are challenging, since the presentation shows great variability. Brain MRI, fundoscopy and audiometry enable the diagnosis. Early treatment with immuno-suppressive medication is crucial and can reduce complications like dementia, loss of visual acuity and hearing. The treatment is empirical and based on the fact, that the histopathology of the syndrome is similar to juvenile dermatomyositis.
这是一例56岁男性Susac综合征患者的病例报告。该综合征是一种罕见的免疫介导的缺血性闭塞性微血管内皮病,累及脑、视网膜和内耳。由于其临床表现差异很大,诊断和治疗颇具挑战性。脑部磁共振成像(MRI)、眼底镜检查和听力测定有助于诊断。早期使用免疫抑制药物治疗至关重要,可减少如痴呆、视力丧失和听力丧失等并发症。治疗是经验性的,基于该综合征的组织病理学与青少年皮肌炎相似这一事实。