IT-DEX 和 B 细胞耗竭治疗一名表现为 NORSE 的抗 GAD 65 自身免疫性脑炎患儿:病例报告。

IF 2.9 4区 医学 Q3 IMMUNOLOGY
Jonathan M. Yarimi , Alexander J. Sandweiss , Karla P. Salazar , Chrissie Massrey , Alexander Ankar , Eyal Muscal , Yi-Chen Lai , Jon A. Cokley , Daniel Davila-Williams , Nikita M. Shukla , Kristen S. Fisher
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引用次数: 0

摘要

新发难治性癫痫(NORSE)是一种破坏性临床症状,通常会导致严重残疾。据报道,鞘内地塞米松(IT-DEX)可改善难治性癫痫状态。我们为您介绍一名 11 岁女性患者,她患有抗 GAD 65 脑炎,表现为 NORSE,对标准抗癫痫药物和一线免疫疗法反应极小。患者在接受利妥昔单抗治疗的同时还接受了 6 次 IT-DEX 治疗,这与随后神经炎症的减轻、癫痫发作的减少以及麻醉输液的断流有关。我们的病例和文献综述表明,IT-DEX 可作为一种早期干预手段,用于治疗各种病因引起的难治性癫痫状态。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

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

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

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.

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来源期刊
Journal of neuroimmunology
Journal of neuroimmunology 医学-免疫学
CiteScore
6.10
自引率
3.00%
发文量
154
审稿时长
37 days
期刊介绍: The Journal of Neuroimmunology affords a forum for the publication of works applying immunologic methodology to the furtherance of the neurological sciences. Studies on all branches of the neurosciences, particularly fundamental and applied neurobiology, neurology, neuropathology, neurochemistry, neurovirology, neuroendocrinology, neuromuscular research, neuropharmacology and psychology, which involve either immunologic methodology (e.g. immunocytochemistry) or fundamental immunology (e.g. antibody and lymphocyte assays), are considered for publication.
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