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霉酚酸酯在儿童拉莫三嗪综合征中的疗效和耐受性。 (注:原文中的“Rasmussen syndrome”可能有误,常见的是“Rasmussen encephalitis”即“拉莫三嗪脑炎”,这里按照给定原文翻译)

Efficacy and tolerability of mycophenolate mofetil in a pediatric Rasmussen syndrome.

作者信息

Orsini A, Costagliola G, Perna D, Esposito M G, Bonfiglio L, Striano P, Peroni D, Consolini R, Bonuccelli A

机构信息

Pediatric Neurology, Pediatric Department, Azienda Ospedaliera Universitaria Pisana, University of Pisa, Italy.

Pediatric Immunology, Pediatric Department, Azienda Ospedaliera Universitaria Pisana, University of Pisa, Italy.

出版信息

Epilepsy Behav Rep. 2019 Oct 21;13:100334. doi: 10.1016/j.ebr.2019.100334. eCollection 2020.

Abstract

Rasmussen syndrome (RS) is a chronic encephalopathy with uncertain etiology and immune-mediated pathogenesis. The only definitive treatment is represented by functional hemispherectomy. We describe the case of a 6.5-year-old female patient who developed several episodes of focal, unilateral clonic seizures. Following laboratory and instrumental investigations, the patient was diagnosed as having RS. A treatment with corticosteroids, intravenous immunoglobulin, and the antiseizure medication (carbamazepine and levetiracetam) did not completely control the seizures. Therefore, the patient was treated with mycophenolate mofetil (MMF), showing a good clinical response, with reduction of the seizures, and stability of the radiological findings. This case suggests the potential utility of MMF in the immune approach to RS.

摘要

拉斯穆森综合征(RS)是一种病因不明且发病机制为免疫介导的慢性脑病。唯一确切的治疗方法是功能性半球切除术。我们描述了一名6.5岁女性患者的病例,该患者出现了数次局灶性、单侧阵挛性癫痫发作。经过实验室和影像学检查,该患者被诊断为患有RS。使用皮质类固醇、静脉注射免疫球蛋白和抗癫痫药物(卡马西平和左乙拉西坦)治疗未能完全控制癫痫发作。因此,该患者接受了霉酚酸酯(MMF)治疗,显示出良好的临床反应,癫痫发作减少,影像学检查结果稳定。该病例提示MMF在RS的免疫治疗方法中具有潜在效用。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/fa01/7044645/b439ccb10be6/gr1.jpg

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