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Akute disseminierte Enzephalomyelitis

Acute disseminated encephalomyelitis

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Zusammenfassung

Die akute disseminierte Enzephalomyelitis (ADEM) ist eine akut auftretende autoimmune demylinisierende Erkrankung der weißen Substanz, die hauptsächlich Gehirn und Rückenmark befällt. Üblicherweise tritt sie nach einer Infektion oder Impfung auf. Die Entwicklung einer fokalen oder multifokalen neurologischen Funktionsstörung ist das Kennzeichen der klinischen Präsentation der ADEM. Lymphozytäre Pleozytose und Eiweißerhöhung sind typische Befunde in der Liquoruntersuchung. Die Magnetresonanztomographie (MRT) ist die Untersuchungsmethode der Wahl. Die ADEM-Läsionen sind typischerweise groß, multipel und asymmetrisch. Sie können in den Groß- und Kleinhirnhemisphären, im Hirnstamm und im Rückenmark lokalisiert sein. Die subkortikale und die zentrale weiße Substanz sind am häufigsten befallen. Weniger häufig ist die graue Substanz der Thalami und der Basalganglien betroffen. Im Gegensatz zur Multiplen Sklerose (MS) ist die Prognose der ADEM im Allgemeinen günstig.

Abstract

Acute disseminated encephalomyelitis (ADEM) is an acute widespread autoimmune demyelinating condition, which principally affects the white matter of the brain and spinal cord. It usually follows an infection or vaccination. The typical presentation is that of multifocal neurologic disturbances accompanied by change in mental status. CSF analysis reveals lymphocytic pleocytosis and elevated protein content, but may also yield normal results. MRI is regarded as the diagnostic imaging modality of choice and typically demonstrates involvement of deep cerebral hemispheric and subcortical white matter as well as lesions in the basal ganglia, gray-white junction, diencephalon, brainstem, cerebellum and spinal cord. Unlike multiple sclerosis (MS), ADEM has a monophasic course and a favorable long-term prognosis.

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Politi, M., Papanagiotou, P., Grunwald, I. et al. Akute disseminierte Enzephalomyelitis. Radiologe 48, 553–559 (2008). https://doi.org/10.1007/s00117-008-1693-y

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