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NMO-Spektrum-Erkrankungen (NMOSE)

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Klinische Neurologie

Part of the book series: Springer Reference Medizin ((SRM))

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Zusammenfassung

Vor allem die Entdeckung des pathogenetisch relevanten Autoantikörpers gegen Aquaporin-4, ein Kanalprotein, welches u. a. auf Astrozyten exprimiert wird, begründet die Definition der Neuromyelitis-optica(NMO)-Spektrum-Erkrankungen (NMOSE) als eine eigenständige Krankheitsentität innerhalb der chronisch-entzündlichen Erkrankungen des Zentralnervensystems. Die diagnostischen Kriterien wurden rezent aktualisiert und beinhalten neben dem Vorhandensein des Aquaporin-4-IgG-Antikörpers auch erweiterte, zuvor eher unbekanntere klinische Manifestationen. Diese klinischen Kriterien gehen über die klassischen Läsionsorte (Optikusneuritis und Myelitis) hinaus und charakterisieren das Erkrankungsspektrum der NMOSE unter Mitbeteiligung zerebraler Strukturen (Area postrema, Hirnstamm, Dienzephalon, präferenziell beteiligte Strukturen des Großhirns). Da hauptsächlich Erkrankungsschübe für die Akkumulation von Behinderung verantwortlich sind und anders als bei der multiplen Sklerose (MS) progressive Erkrankungsverläufe kaum bekannt sind, kommt neben verlaufsmodifizierenden Therapieformen auch Therapiestrategien für akute Erkrankungsschübe (Steroidpulstherapie, Plasmaaustauschverfahren) eine besondere Rolle zu. Für die verlaufsmodifizierende Therapie bestehen maßgebliche Unterschiede zur MS. Eine Vielzahl zugelassener MS-Therapeutika schlug im Bereich der NMOSE fehl und verschlechterte den Verlauf. Wenngleich formelle Zulassungen für die NMOSE fehlen, besteht mittlerweile mit verschiedenen Substanzen ausreichend Erfahrung zur Formulierung von Therapieempfehlungen.

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Authors and Affiliations

  1. Universitätsklinik für Neurologie, Inselspital, Universitätsspital Bern, Freiburgstrasse, 3010, Bern, Schweiz

    Anke Salmen & Andrew Chan

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  1. Anke Salmen
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  2. Andrew Chan
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Correspondence to Anke Salmen .

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Editors and Affiliations

  1. Essen, Germany

    Peter Berlit

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Salmen, A., Chan, A. (2017). NMO-Spektrum-Erkrankungen (NMOSE). In: Berlit, P. (eds) Klinische Neurologie. Springer Reference Medizin. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-44768-0_161-1

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  • DOI: https://doi.org/10.1007/978-3-662-44768-0_161-1

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  • Print ISBN: 978-3-662-44768-0

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