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Neurologie up2date 2020; 3(01): 43-58
DOI: 10.1055/a-0931-2592
Immunvermittelte und erregerbedingte Erkrankungen des ZNS

Neuromyelitis-optica-Spektrum-Erkrankungen – alte und neue Entwicklungen

Lisa Lohmann
,
Luisa Klotz
,
Heinz Wiendl
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Erratum zu: Neuromyelitis-optica-Spektrum-Erkrankungen – alte und neue EntwicklungenNeurologie up2date 2020; 3(01): e1-e1
DOI: 10.1055/a-1134-2373

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.
Fazit

Take Home Message

Die Durchführung der akuten Schubtherapie ist unabhängig vom Serostatus der Patienten. Eine konsequente Therapie und die frühzeitige Eskalation sind entscheidend für das Outcome.

Kernaussagen

  • Neuromyelitis-optica-Spektrum-Erkrankungen (NMOSD) stellen eine seltene, heterogene Gruppe schubförmig oder monophasisch verlaufender chronisch-entzündlicher ZNS-Erkrankungen dar.

  • 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.

  • AQP4-Ak-positive NMOSD sind Astrozythopathien, Myelin-Oligodendrozyten-Glykoprotein (MOG)-Ak-positive NMOSD sind primär demyelinisierende Erkrankungen.

  • Die differenzialdiagnostische Abgrenzung zur Multiplen Sklerose (MS) ist essenziell, da MS-Therapeutika zu einer Exazerbation der Krankheitsaktivität einer NMOSD führen können.

  • NMOSD können mit weiteren Antikörper-vermittelten Erkrankungen assoziiert sein.

  • Die Therapie gliedert sich in Schubtherapie und Schubprophylaxe.

  • Ein früher Therapiebeginn und die konsequente Eskalation der Schubtherapie sind entscheidend.

  • Azathioprin, Mycophenolat Mofetil (MMF) und Rituximab sind etablierte Off-Label-Therapien mit positiven Langzeitdaten.

  • Eculizumab ist das erste zugelassene Medikament für die verlaufsmodifizierende Therapie. Satralizumab und Inebilizumab werden folgen.



Publication History

Article published online:
17 February 2020

© Georg Thieme Verlag KG
Stuttgart · New York

 

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