眼部症状首发Susac综合征1例5年随访  

Five years follow up of a Susac syndrome with initial ophthalmic symptoms

作　　者：古力加乃提·麦麦吐逊 严伟[1] 麦路德木·麦麦吐逊[1] 蒋海山[2] MAIMAITUXUN Gulijianaiti;YAN Wei;MAIMAITUXUN Mailudemu;JIANG Haishan(Department of Neurology,Nanfang Hospital,Southern Medical University,Guangzhou 510515,China)

机构地区：[1]喀什地区第一人民医院神经内科,喀什844000 [2]南方医科大学南方医院神经内科

出　　处：《中国神经精神疾病杂志》2022年第12期754-758,共5页Chinese Journal of Nervous and Mental Diseases

基　　金：新疆维吾尔自治区自然科学基金青年项目(编号:2020D01C012)。

摘　　要：报告1例眼科症状首发Susac综合征患者5年的病情演变及诊治过程。患者,男,29岁。因反复出现双眼视力下降、复视、头痛2年,突发言语不清、右侧肢体无力8 h入院。神经系统查体:计算力减退;双眼视力下降,右侧鼻唇沟变浅,悬雍垂偏左,构音不清,咽反射弱,伸舌偏右;右侧肢体肌力4级,右侧Babinski征(+)。MR示胼胝体雪球样异常信号,眼底造影可见视网膜动脉荧光渗漏。听力五项提示中低频感音性耳聋。诊断:Susac综合征。经糖皮质激素及免疫抑制剂治疗有效。本文结合相关文献的复习,对其易误诊原因,诊断方法及临床转归进行分析讨论,以提高对该病的认识。Here we reported a 5-year fellow-up on a case of a Susac Syndrome(SS) patient who was initially presented with ophthalmic symptoms. The patient was a 29-year-old young man, who had a history of repeated decline of binocular vision, diplopia and headache for 2 years prior to hospital, was admitted to the hospital due to the sudden-onset barylalia and adynamia of right limbs for 8 hours. Neurologic exam showed weaken computing power, decreased vision of both eyes, unclear articulation, weakness of pharyngeal reflex and tongue deviation. Muscle strength of the right limb was grade 4, with positive Babinski sign of right side. T2-weighted MRI showed abnormal snowball like signal within corpus callosum. Moreover, fundus angiography revealed fluorescein leakage of retinal artery, and audiometry analysis indicated middle and low frequency sensorineural hearing loss. Overall, he was diagnosed with Susac syndrome. The condition was improved after glucocorticoid and immunosuppressant treatment. In general, based on the review of relevant literature, we discussed causes of the misdiagnosis of SS, analyzed clinical outcomes and summarized diagnostic methods in this report to increase the understanding of the disease.

关 键 词：SUSAC综合征 视网膜荧光渗漏 伴有视网膜荧光渗漏的神经经系统疾病 视网膜分支动脉闭塞 听觉受损 随访 

分 类 号：R741[医药卫生—神经病学与精神病学]