Department of Neurology, Bielański Hospital, Warsaw, Poland.
Department of Neurology, Faculty of Medical Science, Medical University of Warsaw, Poland.
Neurol Neurochir Pol. 2022;56(2):141-147. doi: 10.5603/PJNNS.a2021.0082. Epub 2022 Jan 5.
Susac's syndrome is a rare microangiopathy affecting small vessels of the retina, inner ear and brain. It is characterised by a triad of symptoms: encephalopathy, visual defects, and sensorineural hearing loss. The disease is probably caused by an autoimmune process. Diagnosis is based on the typical symptoms, brain MRI, and, most importantly, fluorescein angiography. It is important to distinguish between Susac's syndrome and multiple sclerosis or migraine with aura, because misdiagnosis leads to the wrong treatment. To date, no detailed guidelines for the treatment of Susac's syndrome have been developed. Immunosuppression seems to be effective. It must be remembered that early and aggressive treatment is crucial, and that delays in diagnosis, and as a result in treatment implementation, worsen the prognosis.
苏萨克斯综合征是一种罕见的小血管疾病，影响视网膜、内耳和大脑的小血管。其特征为三联征：脑病、视觉缺陷和感觉神经性听力损失。该病可能由自身免疫过程引起。诊断基于典型症状、脑 MRI，最重要的是，荧光素血管造影。重要的是要将苏萨克斯综合征与多发性硬化症或有先兆的偏头痛区分开来，因为误诊会导致错误的治疗。迄今为止，尚未制定苏萨克斯综合征的详细治疗指南。免疫抑制似乎有效。必须记住，早期和积极的治疗至关重要，诊断延迟以及由此导致的治疗实施延迟会使预后恶化。
