Bristol Eye Hospital, Bristol Royal Infirmary NHS Trust, Department of Medical Retina and Uveitis, Bristol Eye Hospital, Bristol, United Kingdom; National Institute for Health Research (NIHR) Biomedical Research Centre at Moorfields Eye Hospital, University College London Institute of Ophthalmology, London, United Kingdom.
Bristol Eye Hospital, Bristol Royal Infirmary NHS Trust, Department of Medical Retina and Uveitis, Bristol Eye Hospital, Bristol, United Kingdom.
Surv Ophthalmol. 2019 Jul-Aug;64(4):477-485. doi: 10.1016/j.survophthal.2019.01.007. Epub 2019 Jan 29.
Susac syndrome is a rare condition presumed to be immune-mediated occlusion of small arterial vasculature principally of the brain, inner ear, and retina. Clinically, the syndrome manifests as a pathognomonic triad of encephalopathy, hearing loss, and branch retinal artery occlusion. Early recognition and diagnosis is important as delayed treatment may be profound and result in deafness, blindness, dementia, and other neurological deficits. The plethora of imaging technology, including magnetic resonance imaging, retinal fluorescein angiography, optical coherence tomography, and optical coherence tomography angiography, allows deeper and more discrete anatomical-physiological correlation of underlying pathology, early diagnosis, and imaging biomarkers for early detection of relapse during follow-up. We highlight the current clinical classification of Susac syndrome, available investigations, treatment, and care pathways.
舒塞综合征是一种罕见的疾病，被认为是小动脉血管（主要是脑、内耳和视网膜）的免疫介导性闭塞。临床上，该综合征表现为脑病、听力损失和视网膜分支动脉阻塞三联征。早期识别和诊断很重要，因为延迟治疗可能会很严重，并导致耳聋、失明、痴呆和其他神经功能缺陷。大量的成像技术，包括磁共振成像、视网膜荧光素血管造影、光学相干断层扫描和光学相干断层扫描血管造影，允许对潜在病理学进行更深层次和更离散的解剖-生理学相关性分析，从而实现早期诊断，并在随访期间通过成像生物标志物来早期发现复发。我们重点介绍了舒塞综合征的当前临床分类、现有检查、治疗和护理途径。
