PMID- 33202462 OWN - NLM STAT- MEDLINE DCOM- 20201119 LR - 20220531 IS - 1439-3999 (Electronic) IS - 0023-2165 (Linking) VI - 237 IP - 11 DP - 2020 Nov TI - [Aquaporin-4 and Myelin Oligodendrocyte Glycoprotein Antibody-Associated Optic Neuritis: Diagnosis and Treatment]. PG - 1290-1305 LID - 10.1055/a-1219-7907 [doi] AB - Optic neuritis (ON) is a frequent manifestation of aquaporin-4 (AQP4) antibody-mediated neuromyelitis optica spectrum disorders (NMOSD) and myelin oligodendrocyte glycoprotein antibody-associated encephalomyelitis (MOG-EM; also termed MOG antibody-associated disorders, MOGAD). The past few years have seen major advances in the diagnosis and treatment of these two relatively new entities: international diagnostic criteria for NMOSD and MOG-EM have been proposed, improved antibody assays developed, and consensus recommendations on the indications and methodology of serological testing published. Very recently, the results of four phase III trials assessing new treatment options for NMOSD have been presented. With eculizumab, a monoclonal antibody inhibiting complement factor C5, for the first time a relapse-preventing long-term treatment for NMOSD - which has so far mostly been treated off-label with rituximab, azathioprine, and other immunosuppressants - has been approved. Data from recent retrospective studies evaluating treatment responses in MOG-ON suggest that rituximab and other immunosuppressants are effective also in this entity. By contrast, many drugs approved for the treatment of multiple sclerosis (MS) have been found to be either ineffective or to cause disease exacerbation (e.g., interferon-beta). Recent studies have shown that not only NMOSD-ON but also MOG-ON usually follows a relapsing course. If left untreated, both disorders can result in severe visual deficiency or blindness, though MOG-ON seems to have a better prognosis overall. Acute attacks are treated with high-dose intravenous methylprednisolone and, in many cases, plasma exchange (PEX) or immunoadsorption (IA). Early use of PEX/IA may prevent persisting visual loss and improve the long-term outcome. Especially MOG-ON has been found to be frequently associated with flare-ups, if steroids are not tapered, and to underlie many cases of "chronic relapsing inflammatory optic neuropathy" (CRION). Both NMOSD-ON and MOG-ON are often associated with simultaneous or consecutive attacks of myelitis and brainstem encephalitis; in contrast to earlier assumptions, supratentorial MRI brain lesions are a common finding and do not preclude the diagnosis. In this article, we review the current knowledge on the clinical presentation, epidemiology, diagnosis, and treatment of these two rare yet important differential diagnoses of both MS-associated ON und idiopathic autoimmune ON. CI - Thieme. All rights reserved. FAU - Wildemann, Brigitte AU - Wildemann B AD - Neurologische Klinik, Universitatsklinikum Heidelberg. FAU - Horstmann, Solveig AU - Horstmann S AD - Neurologische Klinik, Universitatsklinikum Heidelberg. FAU - Korporal-Kuhnke, Mirjam AU - Korporal-Kuhnke M AD - Neurologische Klinik, Universitatsklinikum Heidelberg. FAU - Viehover, Andrea AU - Viehover A AD - Neurologische Klinik, Universitatsklinikum Heidelberg. FAU - Jarius, Sven AU - Jarius S AD - Neurologische Klinik, Universitatsklinikum Heidelberg. LA - ger PT - Journal Article PT - Review TT - Aquaporin-4- und Myelin-Oligodendrozyten-Glykoprotein-Antikorper-assoziierte Optikusneuritis: Diagnose und Therapie. DEP - 20201117 PL - Germany TA - Klin Monbl Augenheilkd JT - Klinische Monatsblatter fur Augenheilkunde JID - 0014133 RN - 0 (Aquaporin 4) RN - 0 (Myelin-Oligodendrocyte Glycoprotein) SB - IM MH - *Aquaporin 4 MH - Humans MH - Myelin-Oligodendrocyte Glycoprotein MH - *Neuromyelitis Optica/diagnosis MH - *Optic Neuritis/diagnosis/therapy MH - Retrospective Studies COIS- B. Wildemann erhielt Forschungsunterstutzung durch das Bundesministerium fur Bildung und Forschung (Klinisches Kompetenznetz Multiple Sklerose), die Dietmar-Hopp-Stiftung und Merck Serono; ausserdem Forschungsunterstutzung durch die Deutsche Forschungsgemeinschaft und die Klaus-Tschira-Stiftung; Forschungsunterstutzung und Honorare fur Vortragstatigkeiten und Reiseunterstutzung von Merck Serono, Sanofi Genzyme, Novartis; und Honorare fur Vortragstatigkeiten und/oder Reiseunterstutzung von Alexion, Bayer, Biogen, Teva. S. Horstmann, S. Jarius, M. Korporal-Kuhnke und A. Viehover geben keine Interessenkonflikte an. EDAT- 2020/11/18 06:00 MHDA- 2020/11/20 06:00 CRDT- 2020/11/17 20:09 PHST- 2020/11/17 20:09 [entrez] PHST- 2020/11/18 06:00 [pubmed] PHST- 2020/11/20 06:00 [medline] AID - 10.1055/a-1219-7907 [doi] PST - ppublish SO - Klin Monbl Augenheilkd. 2020 Nov;237(11):1290-1305. doi: 10.1055/a-1219-7907. Epub 2020 Nov 17.