The role of plasmapheresis in severe acute disseminated encephalomyelitis with clinical findings of transverse myelitis.

IF 1.5 4区 医学 Q3 HEMATOLOGY Therapeutic Apheresis and Dialysis Pub Date : 2024-02-01 Epub Date: 2023-08-30 DOI:10.1111/1744-9987.14059
Olivia W Fjellbirkeland, Wladimir M Szpirt, Malene L Børresen
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Abstract

Introduction: Acute disseminated encephalomyelitis is a rare acute demyelinating disease of the central nervous system (CNS). The pathogenesis remains unclear but is suspected to be autoimmune. High doses of methylprednisolone (HDMP) are currently considered standard of treatment. Plasmapheresis (PE) is typically given in steroid refractory cases. There is currently limited evidence supporting its use in ADEM.

Materials and methods: We report a 16-year-old girl with ADEM who improved rapidly after initiating PE.

Results: The patient presented with acute onset of multifocal CNS symptoms, including encephalopathy, requiring intensive care unit management. Despite HDMP administration, her clinical condition continued to deteriorate. PE was therefore initiated on the same day as HDMP. Her clinical condition improved significantly following the first session. She was extubated and discharged from the intensive care unit the following day.

Conclusion: HDMP combined with PE may be an effective first-line treatment in patients with fulminant ADEM.

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血浆置换在临床表现为横贯性脊髓炎的严重急性播散性脑脊髓炎中的作用。
简介急性播散性脑脊髓炎是一种罕见的中枢神经系统(CNS)急性脱髓鞘疾病。其发病机制尚不清楚,但怀疑与自身免疫有关。目前,大剂量甲基强的松龙(HDMP)被认为是标准治疗方法。类固醇难治性病例通常采用血浆置换术(PE)。目前支持其用于 ADEM 的证据有限:我们报告了一名患有 ADEM 的 16 岁女孩,她在接受 PE 治疗后病情迅速好转:结果:患者急性出现多灶性中枢神经系统症状,包括脑病,需要重症监护室治疗。尽管服用了 HDMP,但她的临床状况仍在继续恶化。因此,在使用 HDMP 的同一天开始使用 PE。第一次治疗后,她的临床状况明显好转。翌日,她被拔除气管,并从重症监护室出院:结论:HDMP联合PE可能是治疗暴发性ADEM患者的有效一线疗法。
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来源期刊
Therapeutic Apheresis and Dialysis
Therapeutic Apheresis and Dialysis 医学-泌尿学与肾脏学
CiteScore
3.00
自引率
10.50%
发文量
166
审稿时长
6-12 weeks
期刊介绍: Therapeutic Apheresis and Dialysis is the official peer-reviewed journal of the International Society for Apheresis, the Japanese Society for Apheresis and the Japanese Society for Dialysis Therapy. The Journal publishes original articles, editorial comments, review articles, case reports, meeting abstracts and Communications information on apheresis and dialysis technologies and treatments.
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