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Neuromyelitis optica

Neuromyelitis optica

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Zusammenfassung

Die Neuromyelitis optica (NMO, Morbus Devic) ist eine seltene entzündlich-demyelinisierende Erkrankung des Zentralnervensystems, die monophasisch oder relapsierend verlaufen kann. Indexereignisse sind Optikusneuritis (uni- oder bilateral) und Myelitis (≥3 Segmente). Eine zerebrale Mitbeteiligung kann vorhanden sein, bleibt aber meist asymptomatisch. Histopathologische Befunde weisen darauf hin, dass die NMO nicht als Variante der Multiplen Sklerose, sondern als eigenständige Entität anzusehen ist. Der kürzliche Nachweis eines hochspezifischen Autoantikörpers gegen Aquaporin-4, einen Wasserkanal, vereinfacht die Diagnosestellung erheblich und charakterisiert die NMO als erste Erkrankung aus dem Formenkreis der entzündlichen Demyelinisierungskrankheiten, für die ein definiertes Autoantigen identifiziert werden konnte. Neuere Therapieansätze wie Plasmaaustausch oder medikamentöse B-Zell-Depletion orientieren sich an der mutmaßlichen humoralen Autoimmunpathogenese der NMO und lassen zukünftig eine bessere Beeinflussbarkeit des vielfach ungünstigen Krankheitsverlaufs erwarten.

Summary

Neuromyelitis optica (NMO, Devic syndrome) is a rare demyelinating disease of the central nervous system which mostly follows a relapsing course. Key features of this disorder include unilateral or bilateral optic neuritis and longitudinally extensive myelitis (≥ three segments). Brain lesions are rarely present at onset. They may however evolve during the course of disease but usually remain asymptomatic. The histopathology of NMO is suggestive of an underlying humoral autoimmune pathomechanism and indicates that NMO is a distinct entity rather than a variant of multiple sclerosis. The recent detection of NMO-specific serum autoantibodies against the water channel aquaporin-4 (Aqp4) is of significant diagnostic relevance and classifies NMO as the first inflammatory demyelinating disorder of the CNS with a defined autoantigen. More recent therapeutic strategies such as plasma exchange or pharmacological B-cell depletion are expected to improve long-term prognosis of NMO.

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  1. Neurosciences Group, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, University of Oxford, OX3 9DS, Oxford, United Kingdom

    S. Jarius

  2. Neurologische Klinik, Sektion Molekulare Neuroimmunologie, Ruprecht-Karls-Universität, Heidelberg, Deutschland

    B. Wildemann

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Jarius, S., Wildemann, B. Neuromyelitis optica. Nervenarzt 78, 1365–1377 (2007). https://doi.org/10.1007/s00115-007-2262-8

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