Gunaratnam Cadric, King Thomas, Moloney Thomas P, Boggild Mike, Goodwin Todd
Department of Neurology, Townsville University Hospital, Townsville 4810, Australia.
School of Medicine, University of Queensland, Brisbane 4006, Australia.
J Surg Case Rep. 2023 Oct 18;2023(10):rjad541. doi: 10.1093/jscr/rjad541. eCollection 2023 Oct.
Susac syndrome (SS) is a rare microangiopathy affecting the precapillary arterioles of the brain, inner ear, and retina. We present a novel case of SS, presenting as acute incomplete bitemporal field loss in addition to temporally spaced neurological and vestibulocochlear symptoms. A 39-year-old female was referred to the ophthalmology clinic with acute incomplete bitemporal hemianopia and worsening hemicrania. History revealed progressive hearing loss, subjective short-term memory impairment, and vertigo temporally spaced over the preceding 12 months. Magnetic resonance brain revealed multiple small colosal lesions and liner 'spoke' lesions. Fundus fluorescein angiography revealed multiple branch retinal artery occlusions in the right eye. Audiometry confirmed bilateral sensorineural hearing loss. Treatment included intravenous corticosteroids and rituximab. This case highlights the importance of early consideration and evaluation of SS in individuals presenting with atypical ocular disturbances, where no clear cause can be elicited, in order to limit the sequelae of disease.
Susac综合征（SS）是一种罕见的微血管病，可累及脑、内耳和视网膜的毛细血管前小动脉。我们报告一例新型SS病例，除了间歇性出现的神经和前庭蜗症状外，还表现为急性不完全双颞侧视野缺损。一名39岁女性因急性不完全双颞侧偏盲和偏头痛加重被转诊至眼科诊所。病史显示，在过去12个月中，她有进行性听力丧失、主观短期记忆障碍以及间歇性眩晕。脑部磁共振成像显示多个小的胼胝体病变和线状“辐条样”病变。眼底荧光血管造影显示右眼有多个视网膜分支动脉阻塞。听力测定证实为双侧感音神经性听力损失。治疗包括静脉注射皮质类固醇和利妥昔单抗。该病例强调了对于出现非典型眼部障碍且无明确病因的个体，早期考虑和评估SS的重要性，以便限制疾病的后遗症。
