Catarsi Eleonora, Pelliccia Veronica, Pizzanelli Chiara, Pesaresi Ilaria, Cosottini Mirco, Migliorini Paola, Tavoni Antonio
Department of Internal Medicine, Immuno-Allergology Unit, University of Pisa, via Roma 67, 56126, Pisa, Italy,
Clin Rheumatol. 2015 Jun;34(6):1149-52. doi: 10.1007/s10067-014-2638-7. Epub 2014 May 3.
Susac's Syndrome (SS) is a rare disease with unknown aetiology due to a microangiopathy affecting the precapillary arterioles of the brain, retina, cochlea and semicircular canals. Neurological manifestations, visual dysfunction and hearing loss represent the classical clinical triad of SS. Diagnosis is confirmed by laboratory investigations, neuroimaging findings, fluoroangiography and inner-ear studies. An early treatment with steroids and immunosuppressors limits the sequelae of disease. We report a case of SS in which the clinical triad occurred in a very short period of time. Brain MRI showed the involvement of cerebellum, this representing a rare neuroradiological finding in SS. A full remission of disease was obtained by using corticosteroids and cyclophosphamide in the acute-subacute phase and methotrexate as maintenance therapy. This latter has never been used before in SS.
苏萨克综合征（SS）是一种病因不明的罕见疾病，由一种影响脑、视网膜、耳蜗和半规管的毛细血管前小动脉的微血管病变引起。神经学表现、视觉功能障碍和听力损失是SS的典型临床三联征。通过实验室检查、神经影像学检查、荧光血管造影和内耳研究来确诊。早期使用类固醇和免疫抑制剂治疗可限制疾病的后遗症。我们报告一例SS病例，其中临床三联征在很短时间内出现。脑部磁共振成像显示小脑受累，这在SS中是一种罕见的神经放射学表现。在急性-亚急性期使用皮质类固醇和环磷酰胺，并使用甲氨蝶呤作为维持治疗，使疾病完全缓解。后者此前从未在SS中使用过。
