急性播散性脑脊髓炎-一种单音疾病。

International review of neurobiology Pub Date : 2025-01-01 Epub Date: 2025-05-16 DOI:10.1016/bs.irn.2025.04.004
Nitin Kumar Singh, Varsha Gupta, Siddhartha Kumar Mishra
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引用次数: 0

摘要

急性播散性脑脊髓炎(ADEM)是一种自身免疫性疾病,通常由感染或免疫活动引起,其特征是中枢神经系统(CNS)的炎症和脱髓鞘。它主要影响大脑和脊髓,通常由免疫反应引发,最常发生在儿童身上,但也可能影响成人。这种疾病通常表现为神经系统症状的快速发作,被认为是单相疾病,这意味着它通常只发生一次。它的发作会导致炎症和脱髓鞘,损害神经信号的传递。虽然确切的机制尚不完全清楚,但分子模仿被认为发挥了作用,其中感染诱导免疫反应错误地靶向与感染因子相似的CNS抗原。尽管知识和治疗方法有所改善,但ADEM仍然难以诊断和治疗。本章涵盖ADEM的诊断、病因、预后和临床方面。它强调免疫系统和中枢神经系统之间的相互作用,个体化治疗计划,以及神经影像学和脑脊液检查在诊断中的作用。有希望的免疫调节药物和定制的药物策略正在开发,以提高患者的结果,即使皮质类固醇仍然是主要的治疗。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Acute disseminated encephalomyelitis - a monophonic illness.

Acute Disseminated Encephalomyelitis (ADEM) is an autoimmune condition frequently caused by infections or immunisation activities and is characterised by inflammation and demyelination in the central nervous system (CNS). It primarily affects the brain and spinal cord, typically triggered by an immune response, and most often occurs in children but can also affect adults. The condition usually presents with a rapid onset of neurological symptoms and is considered a monophasic illness, meaning it generally occurs as a single episode. Its attack leads to inflammation and demyelination, impairing nerve signal transmission. Although the exact mechanism is not fully understood, molecular mimicry is thought to play a role, wherein an infection induces an immune response that mistakenly targets CNS antigens resembling the infectious agent. Despite improvements in knowledge and therapies, ADEM is still difficult to diagnose and treat. This chapter covers ADEM's diagnosis, etiology, prognosis, and clinical aspects. It emphasises the interactions between the immune system and the CNS, the individualised treatment plans, and the role of neuroimaging and cerebrospinal fluid investigations in diagnosis. Promising immunomodulatory medications and customised medicine strategies are being developed to enhance patient outcomes, even if corticosteroids are still the primary therapy.

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