Objective: To assess eyes with neuromyelitis optica (NMO) for morphologic retinal abnormalities utilizing high-definition optical coherence tomography (OCT) imaging.
Methods: In this cross-sectional study, 39 patients with NMO spectrum disorders and 39 age- and sex-matched healthy controls underwent spectral-domain OCT and visual function testing.
Results: Microcystic macular edema (MME) of the inner nuclear layer (INL) was identified in 10 of 39 patients (26%) and was exclusively found in eyes with a history of optic neuritis (ON). MME eyes had lower high- and low-contrast letter-acuity scores (100%: p = 0.002; 2.5%: p = 0.002; 1.25%: p = 0.004), lower peripapillary retinal nerve fiber layer (RNFL) thickness (p = 0.04), lower macular RNFL thickness (p = 0.004), lower ganglion cell layer + inner plexiform layer (GCIP) thickness (p = 0.007), higher INL thickness (p < 0.001), and a greater number of ON episodes (p = 0.008) relative to non-MME eyes with a history of ON. After adjusting for history of multiple ON episodes, these findings remained significant for macular-RNFL thickness (p = 0.03), INL thickness (p < 0.001), and 100% and 2.5% contrast letter-acuity scores (p = 0.008 and p = 0.03, respectively). NMO spectrum eyes without ON history had lower macular RNFL thickness (p = 0.003), GCIP thickness (p = 0.002), outer nuclear layer thickness (p = 0.02), and low-contrast letter-acuity scores (2.5%: p = 0.03; 1.25%: p = 0.002) compared to healthy controls.
Conclusions: We have identified a pattern of retinal morphologic abnormalities in NMO that is associated with severe retinal axonal and neuronal loss and corresponding visual disability. MME may contribute to poor visual outcomes following NMO-associated ON or alternatively represent a marker of ON severity. Additionally, our results support that subclinical involvement of the anterior visual pathway may occur in NMO spectrum disorders.
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The epidemiology and clinical presentation of seropositive neuromyelitis optica spectrum disorder in a US population.
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Departments of Neurology and Ophthalmology, Programs in Neuroscience and Immunology, Anschutz Medical Campus, University of Colorado School of Medicine, Aurora, Colorado, USA.
Objective: To define the epidemiology and clinical presentation of seropositive neuromyelitis optica spectrum disorder (NMOSD) in a large US health system.
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CIEM MS Research Center, Federal University of Minas Gerais Medical School, Belo Horizonte, MG, Brazil.
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View Article and Find Full Text PDFQuantitative Contribution of Clinical Attacks to Residual Disability in Patients With AQP4-Antibody Neuromyelitis Optica Spectrum Disorder.
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From the Nuffield Department of Clinical Neurosciences (B.C., A.F., R.G., M.I.S.L., J.P.), Oxford University Hospitals, United Kingdom; Department of Neurology (B.C.), Tongji Hospital of Tongji Medical College, Huazhong University of Science of Technology, Wuhan, China; University Hospitals Sussex National Health Service Foundation Trust (S.A.C.), Brighton; Centre for Preventive Neurology (R.D.), Wolfson Institute of Population Health, Queen Mary University of London; Queen Square Multiple Sclerosis Centre (Y.H.), UCL Institute of Neurology, Faculty of Brain Sciences, University College London; Department of Paediatric Neurology (Y.H.), Great Ormond Street Hospital for Children, London; Department of Neurology (C. Halfpenny), University Hospital Southampton NHS Foundation Trust; Department of Neurology (C. Hemingway), Great Ormond Street Hospital for Children, London and Institute of Neurology; Department of Neurology (J.C.H.), University of Plymouth Faculty of Health and University Hospitals; Department of Ophthalmology (E.O.S.), King's College Hospital NHS Foundation Trust, London; Department of Neurology (W.R.), St George's University Hospitals NHS Foundation Trust, London; Department of Neurology (R.J.M.), Gloucestershire Hospitals National Health Service Foundation Trust; Department of Neurology (V.W.), King's College Hospital NHS Foundation Trust, London; Department of Neurology (V.W.), Guy's and St Thomas' National Health Service Foundation Trust, London; Department of Paediatric Neurology (S.R.), John Radcliffe Hospital, Oxford; and Neurology Department (R.G.), Wexham Park Hospital, Frimley Foundation Health Trust, Slough, United Kingdom.
Article Synopsis
- The study investigates how clinical attacks contribute to ongoing disability in patients with aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder (AQP4-NMOSD).
- A total of 165 patients were analyzed using disability scores recorded after at least six months post-attack, with findings showing a significant increase in disability scores correlating with the number and type of relapses.
- Results indicated that specific relapse types, particularly the combination of transverse myelitis and optic neuritis, had the most substantial impact on increasing residual disability.
Cerebellar Involvement in Attacks of Aquaporin-4-IgG Positive Neuromyelitis Optica Spectrum Disorder.
Neurol Neuroimmunol Neuroinflamm
January 2025
From the Department of Neurology and Center for Multiple Sclerosis and Autoimmune Neurology (A.D., L.C., J.J.-W.C., B.G.W., S.A.B., S.J.P., E.P.F.), Mayo Clinic College of Medicine, Rochester, MN; Department of Neurosciences (A.D.), Biomedicine, and Movement Sciences, University of Verona, Italy; Department of Radiology (K.N.K.), Mayo Clinic; Department of Ophthalmology (J.J.-W.C.), Mayo Clinic College of Medicine, Rochester, MN; Department of Neurology (D.M.W., C.V.-S.), Mayo Clinic, Scottsdale, AZ; Department of Neurology (B.G.W.), University of Virginia, Charlottesville; Department of Neurology (A.S.L.-C.), Mayo Clinic College of Medicine, Jacksonville, FL; Neurology Unit (E.S.), University Hospital of Sassari, Italy; and Department of Laboratory Medicine and Pathology (S.J.P., E.P.F.), Mayo Clinic College of Medicine, Rochester, MN.
Objectives: To characterize the frequency and clinicoradiologic phenotype of cerebellar involvement in attacks of aquaporin-4-IgG positive neuromyelitis optica spectrum disorder (AQP4+NMOSD) which are incompletely captured in current diagnostic criteria.
Methods: Brain MRI scans from patients with AQP4+NMOSD in the Mayo Clinic database were reviewed, and those with cerebellar T2-hyperintense lesions ≤30 days from attack onset were included for clinical and radiologic characterization.
Results: From 432 patients with AQP4+NMOSD, we identified 17 (4%) with cerebellar attacks.
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