Retinal Changes in Double-Antibody Seronegative Neuromyelitis Optica Spectrum Disorders

Frederike C. Oertel, Hanna G. Zimmermann, Seyedamirhosein Motamedi, Charlotte Bereuter, Luca Magdalena Manthey, Fereshteh Ashtari, Rahele Kafieh, Alireza Dehghani, Mohsen Pourazizi, Lekha Pandit, Anitha D'Cunha, Orhan Aktas, Philipp Albrecht, Marius Ringelstein, Elena H. Martinez-Lapiscina, Bernardo F. Sanchez Dalmau, Pablo Villoslada, Nasrin Asgari, Romain Marignier, Alvaro Cobo-CalvoLetizia Leocani, Marco Pisa, Marta Radaelli, Jacqueline Palace, Adriana Roca-Fernandez, Maria Isabel S. Leite, Srilakshmi Sharma, Jerome De Seze, Thomas Senger, Michael R. Yeaman, Terry J. Smith, Lawrence J. Cook, Alexander U. Brandt, Friedemann Paul*

*Corresponding author for this work

Research output: Contribution to journalJournal articleResearchpeer-review

8 Downloads (Pure)

Abstract

Background and ObjectivesTo systematically describe the clinical picture of double-Antibody seronegative neuromyelitis optica spectrum disorders (DN-NMOSD) with specific emphasis on retinal involvement.MethodsCross-sectional data of 25 people with DN-NMOSD (48 eyes) with and without a history of optic neuritis (ON) were included in this study along with data from 25 people with aquaporin-4 antibody seropositive neuromyelitis optica spectrum disorder (AQP4-NMOSD, 46 eyes) and from 25 healthy controls (HCs, 49 eyes) for comparison. All groups were matched for age and sex and included from the collaborative retrospective study of retinal optical coherence tomography (OCT) in neuromyelitis optica (CROCTINO). Participants underwent OCT with central postprocessing and local neurologic examination and antibody testing. Retinal neurodegeneration was quantified as peripapillary retinal nerve fiber layer thickness (pRNFL) and combined ganglion cell and inner plexiform layer thickness (GCIPL).ResultsThis DN-NMOSD cohort had a history of [median (inter-quartile range)] 6 (5; 9) attacks within their 5 ± 4 years since onset. Myelitis and ON were the most common attack types. In DN-NMOSD eyes after ON, pRNFL (p < 0.001) and GCIPL (p = 0.023) were thinner compared with eyes of HCs. Even after only one ON episode, DN-NMOSD eyes already had considerable neuroaxonal loss compared with HCs. In DN-NMOSD eyes without a history of ON, pRNFL (p = 0.027) and GCIPL (p = 0.022) were also reduced compared with eyes of HCs. However, there was no difference in pRNFL and GCIPL between DN-NMOSD and AQP4-NMOSD for the whole group and for subsets with a history of ON and without a history of ON-as well as between variances of retinal layer thicknesses.DiscussionDN-NMOSD is characterized by severe retinal damage after ON and attack-independent retinal neurodegeneration. Most of the damage occurs during the first ON episode, which highlights the need for better diagnostic markers in DN-NMOSD to facilitate an earlier diagnosis as well as for effective and early treatments. In this study, people with DN-NMOSD presented with homogeneous clinical and imaging findings potentially suggesting a common retinal pathology in these patients.

Original languageEnglish
Article numbere200273
JournalNeurology: Neuroimmunology and NeuroInflammation
Volume11
Issue number5
Number of pages10
ISSN2332-7812
DOIs
Publication statusPublished - 27. Jun 2024

Fingerprint

Dive into the research topics of 'Retinal Changes in Double-Antibody Seronegative Neuromyelitis Optica Spectrum Disorders'. Together they form a unique fingerprint.

Cite this