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DOI: 10.1055/a-1372-9203
Neuromyelitis-optica-Spektrum-Erkrankungen – alte und neue Entwicklungen
Die Entwicklung der letzten 15 Jahre hat die Diagnostik und Therapie der Neuromyelitis-optica-Spektrum-Erkrankungen (NMOSD) maßgeblich geprägt – von der Entdeckung der Aquaporin-4-Antikörper über die Differenzierung der diagnostischen Kriterien bis zur kürzlichen Zulassung von Eculizumab als erste verlaufsmodifizierende Therapie. Dieser Artikel soll einen Überblick über die bisherigen Erkenntnisse und zukünftigen Therapiemöglichkeiten vermitteln.
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Neuromyelitis-optica-Spektrum-Erkrankungen (NMOSD) stellen eine seltene, heterogene Gruppe schubförmig oder monophasisch verlaufender chronisch-entzündlicher ZNS Erkrankungen dar.
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Die Diagnosestellung ist abhängig vom Aquaporin-4 (AQP4)-Ak-Serostatus. Die 6 Kardinalsymptome sind: Neuritis n. optici, akute Myelitis, Area-postrema-Syndrom, akutes Hirnstammsyndrom, akutes Zwischenhirnsyndrom, symptomatisches zerebrales Syndrom.
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AQP4-Ak-positive NMOSD sind Astrozytopathien, Myelin-Oligodendrozyten-Glykoprotein (MOG)-Ak-positive NMOSD sind primär demyelinisierende Erkrankungen.
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Die differenzialdiagnostische Abgrenzung zur Multiplen Sklerose (MS) ist essenziell, da MS-Therapeutika zu einer Exazerbation der Krankheitsaktivität einer NMOSD führen können.
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NMOSD können mit weiteren Antikörper-vermittelten Erkrankungen assoziiert sein.
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Die Therapie gliedert sich in Schubtherapie und Schubprophylaxe.
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Ein früher Therapiebeginn und die konsequente Eskalation der Schubtherapie sind entscheidend.
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Azathioprin, Mycophenolat Mofetil (MMF) und Rituximab sind etablierte Off-Label-Therapien mit positiven Langzeitdaten.
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Eculizumab ist das erste zugelassene Medikament für die verlaufsmodifizierende Therapie. Satralizumab und Inebilizumab werden folgen.
Publication History
Article published online:
17 May 2021
© 2021. Thieme. All rights reserved.
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