Sveinsson Olafur, Kolloch Jens, Träisk Frank, Brundin Lou
Karolinska Universitetssjukhuset - Neurologiska kliniken Stockholm, Sweden Karolinska Universitetssjukhuset - Neurologiska kliniken Stockholm, Sweden.
Karolinska Universitetssjukhuset - Department of Neuroradiology Stockholm, Sweden Karolinska Universitetssjukhuset - Stockholm, Sweden.
Lakartidningen. 2020 Feb 10;117:FSSS.
Susac syndrome is an autoimmune disease characterized by the clinical triad of encephalopathy, branch retinal artery occlusions and sensorineural hearing loss. It most commonly affects young women. Susac syndrome is most likely underdiagnosed, not the least since only 13% have the clinical triad upon presentation. Many are misdiagnosed with multiple sclerosis or another neuroinflammatory entity. Susac syndrome is a microangiopathy affecting the precapillary arterioles causing infarcts of the brain, retina and inner ear. Beside the clinical symptoms, Susac syndrome is diagnosed by typical radiological features on magnetic resonance imaging and branch retinal artery occlusions, which are best evaluated using fluorescein angiography. Early diagnosis and correct immunosuppressive therapy are of utmost importance for clinical improvement and prevention of permanent disability. Diagnosis and treatment of Susac syndrome requires close cooperation between neurologists, radiologists, ophthalmologists and otorhinologists. Here, we present three cases and a review of the literature.
苏萨克综合征是一种自身免疫性疾病,其临床特征为脑病、视网膜分支动脉阻塞和感音神经性听力损失三联征。它最常影响年轻女性。苏萨克综合征很可能未得到充分诊断,尤其是因为只有13%的患者在就诊时具有该临床三联征。许多患者被误诊为多发性硬化症或其他神经炎症性疾病。苏萨克综合征是一种微血管病,影响毛细血管前小动脉,导致脑、视网膜和内耳梗死。除临床症状外,苏萨克综合征通过磁共振成像上的典型放射学特征和视网膜分支动脉阻塞来诊断,荧光素血管造影对其评估效果最佳。早期诊断和正确的免疫抑制治疗对于临床改善和预防永久性残疾至关重要。苏萨克综合征的诊断和治疗需要神经科医生、放射科医生、眼科医生和耳鼻喉科医生密切合作。在此,我们介绍三例病例并对文献进行综述。