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抗 GAD65 自身免疫性脑炎患儿出现 NORSE 表现:一例 IT-DEX 和 B 细胞耗竭病例报告。

IT-DEX and B cell depletion in a child with anti-GAD 65 autoimmune encephalitis presenting as NORSE: A case report.

机构信息

Department of Pediatrics, Division of Pediatric Neurology and Developmental Neuroscience, Baylor College of Medicine and Texas Children's Hospital, 6701 Fannin Street, Ste 1250, Houston, TX 70030, United States of America.

Department of Pediatrics, Division of Pediatric Neurology and Developmental Neuroscience, Baylor College of Medicine and Texas Children's Hospital, 6701 Fannin Street, Ste 1250, Houston, TX 70030, United States of America.

出版信息

J Neuroimmunol. 2024 Oct 15;395:578430. doi: 10.1016/j.jneuroim.2024.578430. Epub 2024 Aug 8.

Abstract

New-onset refractory status epilepticus (NORSE) is a devastating clinical condition that often leads to severe disability. Intrathecal dexamethasone (IT-DEX) has been reported to improve refractory status epilepticus. We present an 11-year-old female with anti-GAD 65 encephalitis presenting as NORSE who had minimal response to standard anti-seizure medications and first-line immunotherapies. The patient received 6 doses of IT-DEX in conjunction with rituximab which correlated with subsequent decreased neuroinflammation, reduced seizure burden and aided in weaning anesthetic infusions. Our case with literature review suggests IT-DEX may be utilized as an early intervention in those with refractory status epilepticus from various etiologies.

摘要

新诊断为难治性癫痫持续状态(NORSE)是一种破坏性的临床情况,常导致严重残疾。鞘内地塞米松(IT-DEX)已被报道可改善难治性癫痫持续状态。我们报告了一例 11 岁女性,患有抗 GABA 脱羧酶 65 脑炎,表现为 NORSE,对标准抗癫痫药物和一线免疫疗法反应甚微。该患者接受了 6 剂 IT-DEX 联合利妥昔单抗治疗,这与随后的神经炎症减少、癫痫发作减少和帮助停用麻醉输注有关。我们的病例结合文献复习表明,IT-DEX 可作为各种病因的难治性癫痫持续状态的早期干预措施。

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