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Akute disseminierte Enzephalomyelitis (ADEM)

Acute disseminated encephalomyelitis (ADEM)

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Intensivmedizin und Notfallmedizin

Summary

Acute disseminated encephalomyelitis (ADEM) is an unusual demyelinating CNS disease. We review the clinical symptoms, findings from radiological and CSF examinations, and current treatment strategies for ADEM. ADEMis usually associated with a precedent infection or vaccination, but may also occur spontaneously. The clinical symptoms are highly variable. CSF analysis reveals a lymphocytic pleocytosis and an elevated protein content, but may also yield normal results. Magnetic resonance imaging shows solitary or multiple lesionswithin the CNS. Most patients improve under therapy with methylprednisolone. After failure of methylprednisolone, immunoglobulins, plasmapheresis, or cytostatic drugs are employed. Unlike in MS, ADEM has a monophasic course and a favorable long-term prognosis. Until now, there are no diagnostic criteria to reliably distinguish ADEM from MS.

Zusammenfassung

In dieser Arbeit geben wir einen Überblick über Diagnostik und Therapie der akuten disseminierten Enzephalomyelitis (ADEM). ADEM tritt häufig nach Infekten oder Impfung auf, kann aber auch spontan vorkommen. Die klinische Symptomatik ist variabel. Der Liquor zeigt meist eine lymphozytäre Pleozytose und einen erhöhten Proteingehalt; Normalbefunde kommen ebenfalls vor. Die Kernspintomographie weist solitäre oder multiple demyelinisierende Läsionen nach, die häufig Kontrastmittel anreichern. Unter Kortisontherapie kommt es bei der Mehrzahl der Patienten rasch zur Remission. Bei Versagen von Kortison können Immunglobuline, Zytostatika und Plasmapherese eingesetzt werden. Im Unterschied zur Multiplen Sklerose (MS) verläuft die Erkrankung monophasisch und hat eine gute Langzeitprognose. Multiphasische Verläufe sind möglich. Bisher gibt es keine diagnostischen Kriterien, die in der akuten Phase eine sichere Differenzierung der ADEM vom ersten Schub einer MS zulassen.

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Authors and Affiliations

  1. Neurologische Klinik, Klinikum Mannheim der Universität Heidelberg, Theodor Kutzer Ufer 1–3, 68167, Mannheim, Germany

    S. Schwarz

  2. Abteilung Neuroradiologie, Klinikum der Universität, Göttingen

    M. Knauth

  3. Neurologische Klinik, Klinikum Heidelberg der Universität, Heidelberg

    B. Storch-Hagenlocher

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  2. M. Knauth
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  3. B. Storch-Hagenlocher
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Correspondence to S. Schwarz.

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Serie: Die Intensivtherapie bei Entzündungen des Nervensystems Herausgegeben von U. Meyding-Lamadé, V. Schuchardt und E. Schmutzhard

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Schwarz, S., Knauth, M. & Storch-Hagenlocher, B. Akute disseminierte Enzephalomyelitis (ADEM). Intensivmed 42, 496–509 (2005). https://doi.org/10.1007/s00390-005-0551-7

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  • DOI: https://doi.org/10.1007/s00390-005-0551-7

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