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首页> 外文期刊>European journal of neurology: the official journal of the European Federation of Neurological Societies >Susac syndrome: Clinical characteristics and treatment in 29 new cases
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Susac syndrome: Clinical characteristics and treatment in 29 new cases

机译：Susac综合征：29例新病例的临床特点和治疗

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Background and purpose: There are few clinical studies on the attempted treatments and outcomes in patients with Susac syndrome (SS) (retinocochleocerebral vasculopathy). Methods: A retrospective review was performed of all patients presenting with SS at the Mayo Clinic in Rochester, Minnesota, USA (1 January 1998-1 October 2011). Results: There were 29 cases of SS (24 women, mean age at presentation, 35years; range, 19-65; full triad of brain, eye, and ear involvement, n=16; mean follow-up time, 29months). Thirty CSF analyses were performed in 27 cases (mean protein 130mg/dl, range 35-268; mean cell count 14, range 1-86). MRI of the brain showed corpus callosal involvement (79%), T2-weighted hyperintensities (93%), and gadolinium enhancement (50%). Average lowest modified Rankin Scale score was 2.5 (median 2, range 0-5). Most patients (93%) received immunosuppressive treatment, with a mean time to treatment of 2months following symptomatic onset. Treatments included intravenous methylprednisolone or dexamethasone (n=23), oral corticosteroids (n=24), plasma exchange (PLEX) (n=9), intravenous immunoglobulin (IVIg) (n=15), cyclophosphamide (n=6), mycophenolate mofetil (n=5), azathioprine (n=2), and rituximab (n=1). Most patients also received an antiplatelet agent (n=21). Improvement or stabilization was noted in eight of 11 cases treated with IVIg in the acute period (three experienced at least partial deterioration) and eight of nine cases of PLEX treatment (one lost to follow up). Conclusions: Susac syndrome may be severe, disabling, and protracted in some patients. PLEX may be an adjunct or alternative therapy for patients who do not experience symptomatic improvement following steroid treatment.
机译：背景与目的：关于Susac综合征（SS）（视网膜脑脊髓血管病）患者尝试的治疗和预后的临床研究很少。方法：对美国明尼苏达州罗切斯特市梅奥诊所所有出现SS的患者进行回顾性研究（1998年1月1日至2011年10月1日）。结果：共有29例SS患者（24名女性，出现时的平均年龄为35岁;范围为19-65岁;大脑，眼睛和耳朵受累的三联症为n = 16;平均随访时间为29个月）。 27例患者进行了30例CSF分析（平均蛋白130mg / dl，范围35-268;平均细胞计数14，范围1-86）。脑部MRI显示showed体受累（79％），T2加权高强度（93％）和g增强（50％）。平均最低修正兰金量表评分为2.5（中位数2，范围0-5）。大多数患者（93％）接受了免疫抑制治疗，平均症状发作后的治疗时间为2个月。治疗方法包括静脉注射甲基强的松龙或地塞米松（n = 23），口服皮质类固醇（n = 24），血浆置换（PLEX）（n = 9），静脉注射免疫球蛋白（IVIg）（n = 15），环磷酰胺（n = 6），霉酚酸酯Mofetil（n = 5），硫唑嘌呤（n = 2）和rituximab（n = 1）。大多数患者还接受了抗血小板药（n = 21）。在急性期接受IVIg治疗的11例患者中有8例得到改善或稳定（3例至少部分恶化），PLEX治疗的9例患者中有8例（随访失败）。结论：Susac综合征在某些患者中可能是严重的，致残的和长期的。对于类固醇治疗后没有症状改善的患者，PLEX可能是辅助治疗或替代治疗。

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