Susac's syndrome: diagnostic challenges.

IF 2.3 Q2 CLINICAL NEUROLOGY PRACTICAL NEUROLOGY Pub Date : 2025-07-14 DOI:10.1136/pn-2024-004462
Khuloud Elsabbagh, Mai Elrayes, Amit Herwadkar, Amira Stylianides, David Gosal, Nazar Sharaf
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Abstract

A 39-year-old woman presented with headaches, slurred speech, facial weakness and brief altered sensations, together with non-specific visual disturbances, unsteadiness and falls. Despite initial treatment with intravenous methylprednisolone, her condition worsened with cognitive decline, confusion, ataxia and incontinence. We diagnosed Susac's syndrome having excluded other causes. Her management was difficult, taking 4 months to achieve remission with corticosteroids, rituximab, cyclophosphamide and intravenous immunoglobulin (Ig). She continues to be on aspirin, intravenous Ig and mycophenolate mofetil to maintain remission, with cyclophosphamide planned for possible relapses.

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苏萨克综合征:诊断挑战。
一名39岁女性,表现为头痛、言语不清、面部无力和短暂的感觉改变,同时伴有非特异性视觉障碍、不稳定和跌倒。尽管最初接受静脉注射甲基强的松龙治疗,但她的病情恶化,出现认知能力下降、意识不清、共济失调和尿失禁。在排除了其他原因后,我们诊断出了苏萨克综合征。她的治疗很困难,用了4个月的时间皮质类固醇、利妥昔单抗、环磷酰胺和静脉注射免疫球蛋白(Ig)才缓解。她继续服用阿司匹林、静脉注射Ig和霉酚酸酯以维持缓解,并计划使用环磷酰胺治疗可能的复发。
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来源期刊
PRACTICAL NEUROLOGY
PRACTICAL NEUROLOGY Medicine-Neurology (clinical)
CiteScore
3.70
自引率
3.60%
发文量
113
期刊介绍: The essential point of Practical Neurology is that it is practical in the sense of being useful for everyone who sees neurological patients and who wants to keep up to date, and safe, in managing them. In other words this is a journal for jobbing neurologists - which most of us are for at least part of our time - who plough through the tension headaches and funny turns week in and week out. Primary research literature potentially relevant to routine clinical practice is far too much for any neurologist to read, let alone understand, critically appraise and assimilate. Therefore, if research is to influence clinical practice appropriately and quickly it has to be digested and provided to neurologists in an informative and convenient way.
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