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Retinal Optical Coherence Tomography in Neuromyelitis Optica
Frederike Cosima Oertel1, Svenja Specovius1, Hanna G Zimmermann1
1From the Experimental and Clinical Research Center (F.C.O., Svenja Specovius, H.G.Z., C.C., S.M., C.B., A.U.B., F.P.), Max Delbrück Center for Molecular Medicine and Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Germany: NeuroCure Clinical Research Center (F.C.O., Svenja Specovius, H.G.Z., C.C., S.M., C.B., A.U.B., F.P.), Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, Germany; Department of Neurology (F.C.O., A.J.G.), University of California San Francisco, CA; Department of Pediatrics (L.C.), University of Utah, Salt Lake City; CIEM MS Research Center (M.A.L.P., M.A.F.), University of Minas Gerais, Medical School, Belo Horizonte, Brazil; Department of Neurology (H.J.K., J.-W.H.), National Cancer Center, Goyang, Republic of Korea; Department of Neurology (J.P., A.R.-F., M.I.L.), and Department of Ophthalmology (Srilakshmi Sharma), and Department of Ophthalmology (Srilakshmi Sharma), Oxford University Hospitals, National Health Service Trust, UK; Kashani MS Center (F.A.), School of Advanced Technologies in Medicine and Medical Image and Signal Processing Research Center (R.K.), Department of Ophthalmology, Isfahan Eye Research Center (A.D., Mohsen Pourazizi), Isfahan University of Medical Sciences, Iran; Department of Neurology (L.P., A.D'C.), KS Hegde Medical Academy, Nitte University, Mangalore, India; Department of Neurology (O.A., Marius Ringelstein, P.A.), Medical Faculty, Heinrich Heine University Düsseldorf, Germany; Swedish Neuroscience Institute Neuro-Ophthalmology (E.M., C.T.), Seattle, WA; Experimental Neurophysiology Unit (L.L., Marco Pisa, Marta Radaelli), Institute of Experimental Neurology (INSPE) Scientific Institute Hospital San Raffaele and University Vita-Salute San Raffaele, Milan, Italy; Hospital Clinic of Barcelona-Institut d'Investigacions (E.H.M.-L.), Biomèdiques August Pi Sunyer, (IDIBAPS), Spain; Sackler School of Medicine (H.S.-K.), Tel Aviv University, Israel; Neuro-Ophthalmology Division (H.S.-K.), Department of Ophthalmology, Rabin Medical Center, Petah Tikva, Israel; Division of Neurology (Sasitorn Siritho), Department of Medicine, Siriraj Hospital and Bumrungrad International Hospital, Bangkok, Thailand; Neurology Service (J.d.S., Thomas Senger), University Hospital of Strasbourg, France; Institute of Clinical Neuroimmunology (J.H.), Biomedical Center and University Hospital, Ludwig-Maximilians Universitaet Muenchen, Munich, Germany; Neurology (R.M., A.C.C.), Multiple Sclerosis, Myelin Disorders and Neuroinflammation, Pierre Wertheimer Neurological Hospital, Hospices Civils de Lyon, France; Centre d'Esclerosi Múltiple de Catalunya (Cemcat) (A.C.C.), Department of Neurology/Neuroimmunology, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Spain; Department of Neurology and Neurosurgery (D.B., I.M.T.), Escola Paulista de Medicina, Universidade Federal de São Paulo, Brazil; Departments of Neurology (N.A.), Slagelse Hospitals, Institute of Regional Health Research, University of Southern Denmark, Odense; Institute of Regional Health Research (N.A., K.S.), University of Southern Denmark, Odense; Department of Neurology (A.A., U.T.), and Department of Ophthalmology (R.Y.), Cerrahpasa Medical Faculty, Istanbul University, Turkey; The Walton Centre for Neurology and Neurosurgery (A.J., S.H.), Liverpool, UK; The Cleveland Clinic Abu Dhabi (A.J.), United Arab Emirates; NYU Multiple Sclerosis Comprehensive Care Center (Z.R., A.R.), Department of Neurology, NYU School of Medicine, New York; Department of Neurology (Y.M.-D.), University of Michigan Medical School, Ann Arbor; Department of Neurology (I.S.C.), Hospital Clínico de Maracaibo, Venezuela; Moorfield's Eye Hospital (A.P.), University College London, UK; Department of Medicine (M.R.Y.), Los Angeles Biomedical Research Institute at Harbor-University of California at Los Angeles (UCLA) Medical Center, Torrance, CA, United States of America; Department of Medicine (M.R.Y.), David Geffen School of Medicine at UCLA, Los Angeles, CA, United States of America; Departments of Ophthalmology and Visual Sciences (Terry Smith), Kellogg Eye Center, University of Michigan Medical School, Ann Arbor, United States of America; Division of Metabolism (Terry Smith), Endocrine and Diabetes, Department of Internal Medicine, University of Michigan Medical School, Ann Arbor; Department of Neurology (A.U.B.), University of California, Irvine; and Department of Neurology (F.P.), Charité-Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Germany.
Neuromyelitis optica spectrum disorder (NMOSD) with optic nerve (ON) attacks causes significant retinal damage. Preventing attacks is crucial to avoid vision loss and neuroaxonal damage in AQP4-IgG-seropositive patients.
Area of Science:
- Neuro-ophthalmology
- Immunology
- Neuroscience
Background:
- Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune disease targeting aquaporin-4 (AQP4-IgG).
- Previous studies on optic nerve and retinal damage in NMOSD cohorts were limited by small sample sizes and heterogeneity.
- This study investigates optic nerve and retinal damage in a large international cohort of AQP4-IgG-seropositive NMOSD patients.
Purpose of the Study:
- To determine the extent of optic nerve and retinal damage in AQP4-IgG-seropositive NMOSD patients.
- To compare retinal damage in patients with and without a history of optic nerve attacks.
- To assess the impact of previous optic nerve attacks on retinal neuroaxonal damage.
Main Methods:
- A cross-sectional study involving 283 AQP4-IgG-seropositive NMOSD patients and 72 healthy controls (HCs).
- Retrospective data collection from 22 centers using optical coherence tomography (OCT).
- Primary outcome: thickness of the ganglion cell and inner plexiform (GCIP) layer; secondary outcomes: peripapillary retinal nerve fiber layer (pRNFL) thickness and visual acuity (VA).
Main Results:
- Eyes with optic nerve (ON) attacks (NMOSD-ON) showed significantly reduced GCIP layer thickness compared to HCs (57.4 ± 12.2 μm vs. 81.4 ± 5.7 μm).
- GCIP layer loss was more pronounced after the first ON attack.
- NMOSD patients without ON attacks (NMOSD-NON) also exhibited reduced GCIP layer thickness but not pRNFL thickness compared to HCs.
Conclusions:
- Attack prevention is critical to avoid severe neuroaxonal damage and vision loss in NMOSD.
- Therapies to mitigate attack-related damage, especially during the first attack, are needed.
- Mild neuroaxonal changes in ON-unaffected eyes may result from contralateral attacks and require further investigation.

