Neurol Neuroimmunol Neuroinflamm. 2021 Sep 15;8(6):e1068. doi: 10.1212/NXI.0000000000001068. Print 2021 Nov.
Frederike Cosima Oertel, Svenja Specovius, Hanna G Zimmermann, Claudia Chien, Seyedamirhosein Motamedi, Charlotte Bereuter, Lawrence Cook, Marco Aurélio Lana Peixoto, Mariana Andrade Fontanelle, Ho Jin Kim, Jae-Won Hyun, Jacqueline Palace, Adriana Roca-Fernandez, Maria Isabel Leite, Srilakshmi Sharma, Fereshteh Ashtari, Rahele Kafieh, Alireza Dehghani, Mohsen Pourazizi, Lekha Pandit, Anitha D’Cunha, Orhan Aktas, Marius Ringelstein, Philipp Albrecht, Eugene May, Caryl Tongco, Letizia Leocani, Marco Pisa, Marta Radaelli, Elena H Martinez-Lapiscina, Hadas Stiebel-Kalish, Sasitorn Siritho, Jérome de Seze, Thomas Senger, Joachim Havla, Romain Marignier, Alvaro Cobo Calvo, Denis Bichuetti, Ivan Maynart Tavares, Nasrin Asgari, Kerstin Soelberg, Ayse Altintas, Rengin Yildirim, Uygur Tanriverdi, Anu Jacob, Saif Huda, Zoe Rimler, Allyson Reid, Yang Mao-Draayer, Ibis Soto de Castillo, Axel Petzold, Ari J Green, Michael R Yeaman, Terry Smith, Alexander U Brandt, Friedemann Paul
Abstract
Background and objectives: To determine optic nerve and retinal damage in aquaporin-4 antibody (AQP4-IgG)-seropositive neuromyelitis optica spectrum disorders (NMOSD) in a large international cohort after previous studies have been limited by small and heterogeneous cohorts.
Methods: The cross-sectional Collaborative Retrospective Study on retinal optical coherence tomography (OCT) in neuromyelitis optica collected retrospective data from 22 centers. Of 653 screened participants, we included 283 AQP4-IgG-seropositive patients with NMOSD and 72 healthy controls (HCs). Participants underwent OCT with central reading including quality control and intraretinal segmentation. The primary outcome was thickness of combined ganglion cell and inner plexiform (GCIP) layer; secondary outcomes were thickness of peripapillary retinal nerve fiber layer (pRNFL) and visual acuity (VA).
Results: Eyes with ON (NMOSD-ON, N = 260) or without ON (NMOSD-NON, N = 241) were assessed compared with HCs (N = 136). In NMOSD-ON, GCIP layer (57.4 ± 12.2 μm) was reduced compared with HC (GCIP layer: 81.4 ± 5.7 μm, p < 0.001). GCIP layer loss (-22.7 μm) after the first ON was higher than after the next (-3.5 μm) and subsequent episodes. pRNFL observations were similar. NMOSD-NON exhibited reduced GCIP layer but not pRNFL compared with HC. VA was greatly reduced in NMOSD-ON compared with HC eyes, but did not differ between NMOSD-NON and HC.
Discussion: Our results emphasize that attack prevention is key to avoid severe neuroaxonal damage and vision loss caused by ON in NMOSD. Therapies ameliorating attack-related damage, especially during a first attack, are an unmet clinical need. Mild signs of neuroaxonal changes without apparent vision loss in ON-unaffected eyes might be solely due to contralateral ON attacks and do not suggest clinically relevant progression but need further investigation.
Copyright © 2021 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.