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Akute disseminierte Enzephalomyelitis

Pathogenese, Diagnose, Behandlung und Prognose

Acute disseminated encephalomyelitis

Pathogenesis, diagnosis, treatment, and prognosis

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Zusammenfassung

Die akute disseminierte Enzephalomyelitis (ADEM) ist typischerweise eine monophasische demyelinisierende Erkrankung des zentralen Nervensystems (ZNS), von der am häufigsten Kinder betroffen sind. Das klinische Bild dieser Erkrankung manifestiert sich meist in Folge einer Infektion oder Impfung. Diesbezüglich sind zahlreiche virale und bakterielle Krankheitserreger sowie Impfstoffe mit der ADEM assoziiert worden. Untersuchungen in Tiermodellen haben zudem gezeigt, dass vermutlich sowohl primäre als auch sekundäre Immunantworten an der Entstehung dieser entzündlichen demyelinisierenden ZNS-Erkrankung beteiligt sind. Die Diagnose einer ADEM ist wahrscheinlich, wenn ein zeitlicher Zusammenhang zwischen einer infektiösen Erkrankung bzw. Impfung sowie dem Auftreten subakuter, im ZNS lokalisierter Komplikationen determiniert werden kann. In der Bildgebung des Gehirns mittels Magnetresonanztomographie (MRT) finden sich in der Regel ausgedehnte, multifokale Marklagerveränderungen. Während im Liquor meist eine Pleozytose und Proteinerhöhung nachweisbar sind, sind oligoklonale Banden nicht immer vorhanden. Die Behandlung der ADEM erfolgt in der Regel mit antiinflammatorischen und immunsuppressiven Medikamenten, und die Prognose ist im Allgemeinen gut.

Summary

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.

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Authors and Affiliations

  1. Neurologische Klinik, Heinrich-Heine-Universität Düsseldorf

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

  2. Hertie-Institut für Klinische Hirnforschung, Abteilung für Allgemeine Neurologie, Eberhard-Karls-Universität Tübingen

    H. Wiendl

  3. Neurologische Klinik, Heinrich-Heine-Universität Düsseldorf, Moorenstraße 5, 40225, Düsseldorf, Deutschland

    O. Stüve

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  1. O. Stüve
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  2. S. Nessler
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Stüve, O., Nessler, S., Hartung, H.P. et al. Akute disseminierte Enzephalomyelitis . Nervenarzt 76, 701–707 (2005). https://doi.org/10.1007/s00115-004-1842-0

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