{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T07:33:28Z","timestamp":1792740808645,"version":"4.5.0"},"reference-count":84,"publisher":"Georg Thieme Verlag KG","issue":"11","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Klin Monbl Augenheilkd"],"published-print":{"date-parts":[[2020,11]]},"abstract":"<jats:title>Zusammenfassung<\/jats:title><jats:p>Die Optikusneuritis (ON) ist vielfach die erste Manifestation einer AQP4-Antik\u00f6rper-vermittelten NMOSD (AQP4: Aquaporin-4, NMOSD: Neuromyelitis-optica-Spektrum-Erkrankung, Engl.: neuromyelitis optica spectrum disorders) oder einer Myelin-Oligodendrozyten-Glykoprotein-Antik\u00f6rper-assoziierten Enzephalomyelitis (MOG-EM; auch MOG antibody associated disorders, MOGAD). F\u00fcr beide Erkrankungen wurden in den vergangenen Jahren internationale Diagnosekriterien und Empfehlungen zu Indikation und Methodik der serologischen Testung vorgelegt. Seit Kurzem liegen zudem Ergebnisse aus 4 gro\u00dfen, internationalen Phase-III-Studien zur Behandlung der NMOSD vor. Mit dem den Komplementfaktor C5 blockierenden monoklonalen Antik\u00f6rper Eculizumab wurde 2019 erstmalig ein Medikament zur Langzeitbehandlung der NMOSD, die bislang vornehmlich Off-Label mit Rituximab, Azathioprin und anderen Immunsuppressiva erfolgt, auf dem europ\u00e4ischen Markt zugelassen. F\u00fcr die erst vor wenigen Jahren\n\t\t\t\t\terstbeschriebene MOG-EM stehen inzwischen Daten aus mehreren retrospektiven Studien zur Verf\u00fcgung, die eine Wirksamkeit von Rituximab und anderen Immunsuppressiva in der Schubprophylaxe auch in dieser Indikation nahelegen. Viele der zur Therapie der MS zugelassenen Medikamente sind entweder unwirksam oder k\u00f6nnen, wie z.\u200aB. Interferon-\u03b2, eine Verschlechterung des Krankheitsverlaufes bewirken. Beide Erkrankungen werden im Akutstadium mit hochdosierten Glukokortikoiden und Plasmapherese oder Immunadsorption behandelt. Diese Behandlung sollte m\u00f6glichst rasch nach Symptombeginn eingeleitet werden. Insbesondere die MOG-EM ist durch eine oft ausgepr\u00e4gte Steroidabh\u00e4ngigkeit gekennzeichnet, die ein langsames Ausschleichen der Steroidtherapie erfordert, und schlie\u00dft viele F\u00e4lle der bislang meist als \u201eidiopathisch\u201c klassifizierten \u201echronic relapsing inflammatory optic neuropathy\u201c (CRION) ein. Unbehandelt kann sowohl die NMOSD- als auch die MOG-EM-assoziierte ON zu schweren,\n\t\t\t\t\tpersistierenden und oft bilateralen Visuseinschr\u00e4nkungen bis hin zur Erblindung f\u00fchren. Beide Erkrankungen verlaufen meist relapsierend. Neben den Sehnerven sind h\u00e4ufig das Myelon sowie der Hirnstamm und, vor allem bei NMO-Patienten, das Dienzephalon betroffen; supratentorielle Hirnl\u00e4sionen im kranialen MRT sind, anders als fr\u00fcher gedacht, kein Ausschlusskriterium, sondern h\u00e4ufig. In der vorliegenden Arbeit geben wir einen \u00dcberblick \u00fcber Klinik, Diagnostik und Therapie dieser beiden wichtigen Differenzialdiagnosen der MS-assoziierten und idiopathischen ON.<\/jats:p>","DOI":"10.1055\/a-1219-7907","type":"journal-article","created":{"date-parts":[[2020,11,18]],"date-time":"2020-11-18T03:50:22Z","timestamp":1605671422000},"page":"1290-1305","source":"Crossref","is-referenced-by-count":8,"title":["Aquaporin-4- und Myelin-Oligodendrozyten-Glykoprotein-Antik\u00f6rper-assoziierte Optikusneuritis: Diagnose und Therapie"],"prefix":"10.1055","volume":"237","author":[{"given":"Brigitte","family":"Wildemann","sequence":"additional","affiliation":[{"name":"Neurologische Klinik, Universit\u00e4tsklinikum Heidelberg"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Solveig","family":"Horstmann","sequence":"additional","affiliation":[{"name":"Neurologische Klinik, Universit\u00e4tsklinikum Heidelberg"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mirjam","family":"Korporal-Kuhnke","sequence":"additional","affiliation":[{"name":"Neurologische Klinik, Universit\u00e4tsklinikum Heidelberg"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrea","family":"Vieh\u00f6ver","sequence":"additional","affiliation":[{"name":"Neurologische Klinik, Universit\u00e4tsklinikum Heidelberg"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sven","family":"Jarius","sequence":"additional","affiliation":[{"name":"Neurologische Klinik, Universit\u00e4tsklinikum Heidelberg"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"194","published-online":{"date-parts":[[2020,11,17]]},"reference":[{"key":"ref1","doi-asserted-by":"crossref","first-page":"195","DOI":"10.2147\/EB.S54131","article-title":"Optic neuritis as an early sign of multiple sclerosis","volume":"8","author":"N Kale","year":"2016","journal-title":"Eye Brain"},{"key":"ref3","doi-asserted-by":"crossref","first-page":"661","DOI":"10.1111\/bpa.12084","article-title":"Aquaporin-4 antibodies (NMO-IgG) as a serological marker of neuromyelitis optica: a critical review of the literature","volume":"23","author":"S Jarius","year":"2013","journal-title":"Brain Pathol"},{"key":"ref4","doi-asserted-by":"publisher","DOI":"10.1038\/s41572-020-0214-9","article-title":"Neuromyelitis optica","author":"S Jarius","year":"2020","journal-title":"Nat Rev Dis Primers"},{"key":"ref5","doi-asserted-by":"crossref","first-page":"8","DOI":"10.1186\/1742-2094-10-8","article-title":"The history of neuromyelitis optica","volume":"10","author":"S Jarius","year":"2013","journal-title":"J Neuroinflammation"},{"key":"ref6","doi-asserted-by":"crossref","first-page":"280","DOI":"10.1186\/s12974-019-1594-1","article-title":"The history of neuromyelitis optica. 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