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首页> 外文期刊>eNeurologicalSci >Exquisite response to intravenous immunoglobulin in Susac syndrome during pregnancy
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Exquisite response to intravenous immunoglobulin in Susac syndrome during pregnancy

机译：Susac综合征对妊娠期静脉注射免疫球蛋白的反应良好

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Introduction From its initial report on two female patients in 1979 by J.O. Susac, Susac syndrome (SuS) or SICRET (small infarctions of cochlear, retinal and encephalic tissue) has persisted as an elusive entity. To date the available evidence for its treatment is based on case reports and case series. The largest systematic review described only 304 reported cases since the 1970s. Here we presented the first reported case to our knowledge in Mexican population and the unusual presentation in a pregnant patient. Case presentation A 34-year-old Hispanic woman was brought to the ER in our hospital for apathy and behavioral changes. Upon arrival at the ER, her husband described a one-month history of behavioral changes with apathy, progressive abulia, visuospatial disorientation, and gait deterioration. The initial lab test shows no significance except by a positive qualitative hCG. An MRI was obtained and showed hyperintense periventricular white matter lesions in T2 and FLAIR sequences also involving bilateral basal ganglia and with predominant affection of the corpus callosum, in addition to infratentorial cerebellar lesions. After treatment with intravenous immunoglobulins a marked and prompt clinical and radiological improvement was observed. Conclusion SuS is still an elusive disease. To date, no definitive score or clinical feature can predict the outcome of the disease. The presentation during pregnancy is also rare and therefore the optimal treatment and the prognosis is unknown. We hope that this article will serve as a foundation for future research.
机译：引言J.O.于1979年首次报告两名女性患者。 Susac，Susac综合征（SuS）或SICRET（耳蜗，视网膜和脑组织的小梗塞）一直作为一种难以捉摸的实体而持续存在。迄今为止，其治疗的可用证据是基于病例报告和病例系列的。自1970年代以来，最大的系统评价仅描述了304例报告病例。在这里，我们介绍了我们所知的墨西哥人群中第一个报告的病例，以及孕妇中的异常病例。病例介绍一名34岁的西班牙裔妇女因无动于衷和行为改变被带到我们医院的急诊室。到达急诊室后，她的丈夫描述了一个月的行为改变病史，包括冷漠，进行性健忘，视觉空间迷失方向和步态恶化。最初的实验室测试除hCG阳性外没有其他意义。进行了MRI检查，显示T2和FLAIR序列中的高强度脑室周围白质病变，还累及双侧基底神经节，除in下小脑病变外，主要累及call体。静脉注射免疫球蛋白治疗后，观察到明显且迅速的临床和放射学改善。结论SuS仍然是一种难以捉摸的疾病。迄今为止，尚无明确的评分或临床特征可以预测疾病的结果。怀孕期间的表现也不多见，因此最佳治疗和预后尚不清楚。我们希望本文将为将来的研究打下基础。

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