Susac syndrome. A differential diagnosis for demyelination.

Neurosciences (Riyadh, Saudi Arabia) Pub Date : 2013-01-01
Surjith Vattoth, Christopher J Compton, Glenn H Roberson, Michael S Vaphiades
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Abstract

Susac syndrome is a microangiopathy of unknown origin, probably autoimmune, affecting capillaries and precapillary arterioles of the brain, retina, and inner ear. It is often misdiagnosed as acute disseminated encephalomyelitis or multiple sclerosis. We report the case of a 25-year-old male with Susac syndrome who developed the clinical triad of encephalopathy, visual and hearing problems over the course of a year. The characteristic MRI findings including central corpus callosal involvement and brain infarctions were supported by branch retinal arterial/arteriolar occlusions on fluorescein retinal angiography. Most patients with Susac syndrome will not have the complete clinical triad initially. A very high index of suspicion is required by the clinician and radiologist in patients with any component of the clinical triad so as not to misdiagnose the MRI findings for demyelination. Even if initial ophthalmologic examinations are normal, these patients should be followed up for later development of branch retinal artery occlusions.

Susac综合症。脱髓鞘的鉴别诊断。
Susac综合征是一种病因不明的微血管疾病,可能是自身免疫性疾病,影响大脑、视网膜和内耳的毛细血管和毛细血管前小动脉。常误诊为急性播散性脑脊髓炎或多发性硬化症。我们报告的情况下,25岁的男性与苏萨克综合征谁发展的临床三合一脑病,视觉和听力问题的过程中一年。其特征性MRI表现包括中央胼胝体受累和脑梗死,荧光素视网膜血管造影显示视网膜分支动脉/小动脉闭塞。大多数Susac综合征患者最初不会有完整的临床三联征。临床医生和放射科医生对患有临床三联征的患者都需要高度的怀疑,以免误诊脱髓鞘的MRI表现。即使最初的眼科检查是正常的,这些患者也应随访,以了解视网膜分支动脉闭塞的后期发展情况。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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