Bodaghi B, Wechsler B, Adam R, Galanaud D, Fardeau C, Papo T, LeHoang P, Piette J-C
Service d'Ophtalmologie, CHU Pitié-Salpêtrière, Paris.
Rev Neurol (Paris). 2005 Dec;161(12 Pt 1):1221-4. doi: 10.1016/s0035-3787(05)85196-3.
Susac's syndrome consists of the clinical triad of cerebral microangiopathy, branch retinal artery occlusions, and hearing loss. The pathogenesis of the disease remains unknown. The severity of retinal vasculopathy remains variable.
A 57-year-old Caucasian woman presented in 1999 for the diagnostic and therapeutic management of a severe and complete form of the disease. Fluorescein angiography and cerebral MRI were contributive in this case and confirmed the diagnosis. A severe unilateral visual loss occurred one month after the initiation of systemic corticosteroids. Funduscopy disclosed a temporal retinal artery occlusion with major ischemia. Therefore, cyclophosphamide and anticoagulants were added to corticosteroids and total visual recovery was achieved within 2 weeks without any relapse after a follow-up period of 54 months.
Therapeutic management of Susac's syndrome is still controversial. Major immunosuppressive regimens are mandatory in the face of severe visual loss associated with central or branch retinal artery occlusions. The final prognosis of the disease seems favorable in the absence of prolonged and symptomatic retinal ischemia.
Susac综合征由脑微血管病、视网膜分支动脉阻塞和听力丧失这一临床三联征组成。该病的发病机制尚不清楚。视网膜血管病变的严重程度各不相同。
一名57岁的白种女性于1999年就诊，以诊断和治疗该疾病的严重完全型。荧光素血管造影和脑部磁共振成像在此病例中发挥了作用并确诊。全身使用皮质类固醇激素1个月后出现严重的单眼视力丧失。眼底检查发现颞侧视网膜动脉阻塞并伴有严重缺血。因此，在皮质类固醇激素基础上加用了环磷酰胺和抗凝剂，2周内视力完全恢复，随访54个月无任何复发。
Susac综合征的治疗管理仍存在争议。面对与视网膜中央或分支动脉阻塞相关的严重视力丧失，主要的免疫抑制方案是必要的。在没有长期症状性视网膜缺血的情况下，该病的最终预后似乎良好。
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