Department of Pediatrics and Adolescent Medicine, Copenhagen, Denmark.
Department of Nephrology, Plasmapheresis Services, Copenhagen, Denmark.
Ther Apher Dial. 2024 Feb;28(1):119-124. doi: 10.1111/1744-9987.14059. Epub 2023 Aug 30.
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.
We report a 16-year-old girl with ADEM who improved rapidly after initiating PE.
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.
HDMP combined with PE may be an effective first-line treatment in patients with fulminant ADEM.
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。其发病机制仍不清楚，但疑似自身免疫性疾病。目前，大剂量甲基强的松龙（HDMP）被认为是标准治疗方法。在类固醇抵抗的情况下，通常会使用血浆置换（PE）。目前，支持其在 ADEM 中应用的证据有限。
我们报告了一例 ADEM 患者，在开始进行 PE 后迅速改善。
该患者出现了多灶性 CNS 症状的急性发作，包括脑病，需要重症监护病房治疗。尽管给予 HDMP 治疗，但她的临床状况持续恶化。因此，在给予 HDMP 的同一天开始进行 PE。第一次治疗后，她的临床状况明显改善。她于次日拔管并从重症监护病房出院。
在暴发性 ADEM 患者中，HDMP 联合 PE 可能是一种有效的一线治疗方法。
