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DOI: 10.1055/s-0042-124178
NMO-Spektrum-Erkrankungen
NMO Spectrum DisordersPublikationsverlauf
Publikationsdatum:
03. Mai 2017 (online)
![](https://www.thieme-connect.de/media/aktneu/201703/lookinside/thumbnails/0107_10-1055-s-0042-124178-1.jpg)
Zusammenfassung
Neuromyelitis optica-Spektrum-Erkrankungen (NMOSD) stellen eine seltene Subgruppe chronisch-entzündlicher ZNS-Erkrankungen dar. Trotz heterogener Verläufe im Hinblick auf die Krankheitsaktivität ist die Behinderungsakkumulation häufig aufgrund der Schwere einzelner Schubereignisse sehr stark ausgeprägt. Nach Revision der Diagnosekriterien 2015 wurde der Begriff der NMO verlassen und stattdessen der Begriff NMOSD für alle Entitäten vorgeschlagen. Klinisch leiden die Patienten am häufigsten unter Optikusneuritiden und transversen Myelitiden, jedoch sind Manifestationen an verschiedenen Lokalisationen des zentralen Nervensystems möglich (z. B. auch in Hirnstamm oder Hypothalamus). Neben Formen mit und ohne Nachweis von Aquaporin 4-Antikörpern wird seit einiger Zeit auch diskutiert, ob Erkrankungen mit Vorkommen von Antikörpern gegen Myelin-Oligodendrozyten-Glykoprotein (MOG) dem Spektrum der NMOSD zuzurechnen sind oder eine eigene Entität darstellen. Aufgrund der hohen Krankheitsaktivität dieser Erkrankungen ist eine konsequente Therapie indiziert. In Ermangelung zugelassener Therapien werden hierzu konventielle Immunsuppressiva oder monoklonale Antikörper mit antiinflammatorischen Effekten eingesetzt. Seit einiger Zeit sind 4 Substanzen in der fortgeschrittenen klinischen Erprobung.
Abstract
Neuromyelitis optica spectrum disorders (NMOSD) represent a rare subset of chronic-inflammatory diseases of the central nervous system. Despite heterogeneities in disease activity, NMOSD patients have worse disability accumulation compared to MS patients. Revised diagnostic criteria comprise recommendations to abandon the term NMO and to summarize these conditions as NMOSD. Clinical presentation of NMOSD patients in most cases is optic neuritis and transverse myelitis; nevertheless, NMOSD can affect most parts of the central nervous system (e. g. brainstem and hypothalamus). Originally characterised as AQP4-antibody-dependent disease, recently the question was raised whether conditions with the presence of antibodies against myelin-oligodendrocyte glycoprotein (MOG) belong to the family of NMOSD. Due to the severity of the disease with often devastating relapses, systematic therapy is necessary. Usually, immunosuppressants or monoclonal antibodies with anti-inflammatory properties are used. Recently, 4 substances entered clinical testing for treatment of NMOSD.
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