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[急性播散性脑脊髓炎。发病机制、诊断、治疗及预后]

[Acute disseminated encephalomyelitis. Pathogenesis, diagnosis, treatment, and prognosis].

作者信息

Stüve O, Nessler S, Hartung H P, Hemmer B, Wiendl H, Kieseier B C

机构信息

Neurologische Klinik, Heinrich-Heine-Universität Düsseldorf.

出版信息

Nervenarzt. 2005 Jun;76(6):701-7. doi: 10.1007/s00115-004-1842-0.

Abstract

Acute disseminated encephalomyelitis (ADEM) is typically a monophasic, demyelinating disease of the CNS that predominantly affects children. Typically, its clinical symptoms follow an infection or vaccination. In this regard, numerous viral and bacterial pathogens as well as several vaccinations have been associated with ADEM. Studies from animal models suggest that primary and secondary autoimmune responses may contribute to CNS inflammation and demyelination in ADEM. The diagnosis of ADEM is strongly suggested by a close temporal relationship between a viral infection or immunization and the onset of neurologic symptoms, and it is supported by extensive, multifocal, subcortical white-matter disease on brain magnetic resonance imaging. While mild lymphocytic pleocytosis and elevated proteins are detectable in the CSF in ADEM, oligoclonal bands are not always present. Treatment of this disorder consists of anti-inflammatory and immunosuppressive therapies, and the prognosis is generally considered favorable.

摘要

急性播散性脑脊髓炎(ADEM)通常是一种单相性中枢神经系统脱髓鞘疾病,主要影响儿童。通常,其临床症状出现在感染或接种疫苗之后。在这方面,许多病毒和细菌病原体以及几种疫苗接种都与ADEM有关。动物模型研究表明,原发性和继发性自身免疫反应可能导致ADEM中的中枢神经系统炎症和脱髓鞘。病毒感染或免疫接种与神经症状发作之间密切的时间关系强烈提示ADEM的诊断,脑磁共振成像显示广泛、多灶性、皮质下白质病变支持这一诊断。虽然在ADEM患者的脑脊液中可检测到轻度淋巴细胞增多和蛋白质升高,但并不总是出现寡克隆带。这种疾病的治疗包括抗炎和免疫抑制疗法,一般认为预后良好。

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