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Aquaporin-4 Immunoglobulin G-seropositive Neuromyelitis Optica Spectrum Disorder MRI Characteristics: Data Analysis
Claudia Chien1, Vera Cruz E Silva1, Emanuel Geiter1
1From the Experimental and Clinical Research Center, Charité-Universitätsmedizin Berlin, corporate member of Freie Universität Berlin, Humboldt-Universität zu Berlin & Max Delbrück Center for Molecular Medicine in the Helmholtz Association, Lindenberger Weg 80, 13125 Berlin, Germany (C.C., H.Z., A.U.B., F.P.); NeuroCure Clinical Research Ctr (C.C., H.Z., A.U.B., J.W., F.P.), Dept of Psychiatry and Neurosciences, Charité-Universitätsmedizin Berlin, Freie Universität Berlin, Humboldt-Universität zu Berlin, Berlin, Germany (C.C.); Medical Image Analysis Center, Basel, Switzerland (V.C.e.S., E.G., D.M.); Paulista School of Medicine, Dept of Neurology and Neurosurgery (D.B.B.), Dept of Diagnostic Imaging, Universidade Federal de São Paulo, São Paulo, Brazil (M.I.I.); Koc Univ, School of Medicine Neurology Dept and Istanbul Univ, Cerrahpasa School of Medicine, Neurology Dept, Istanbul, Turkey (A.A.); Dept of Neurology, Istanbul Univ, Cerrahpasa Faculty of Medicine, Istanbul, Turkey (U.T.); Div of Neurology, Dept of Medicine, Siriraj Hosp, Mahidol Univ, Bangkok, Thailand (S.S.); Bumrungrad International Hosp, Bangkok, Thailand (S.S.); Center for Advanced Neurologic Research, KS Hegde Medical Academy, Nitte Univ, Mangalore, India (L.P., A.D.); Dept of Neurology, Hosp de S. João, Al. Hernâni Monteiro, Porto, Portugal (M.J.S., R.F.); MS Center at Swedish Neuroscience Inst, Seattle, Wash (P.Q., C.T.); Dept of Neurology and Neuroimmunology Clinic, Rabin Medical Center, Petach Tikva, Israel (I.L.); Sackler Faculty of Medicine & Felsenstein Medical Research Center, Tel Aviv Univ, Tel Aviv, Israel (I.L., H.S.K.); Dept of Radiology, Rabin Medical Center, Beilinson Hosp, Israel, and Sackler Faculty of Medicine, Tel-Aviv Univ, Tel Aviv, Israel (V.K.); Dept of Neurology and Neuroimmunology, Rabin Medical Center, Beilinson Hosp, Israel, and Sackler Faculty of Medicine, Tel-Aviv Univ, Tel Aviv, Israel (M.A.H.); Neuro-Ophthalmology Div, Dept of Ophthalmology, Rabin Medical Center, Petah Tikva, Israel (H.S.K.); Div of Neurology, Univ of Toronto, St Michael's Hosp, Toronto, Canada (D.L.R., L.W.); Mellen Center, Cleveland Clinic, Cleveland, Ohio (D.O.), Dept of Biomedical Engineering, Cleveland Clinic, Cleveland, Ohio (K.N.); Multiple Sclerosis and Neuroimmunology Program, Univ Hosps of Cleveland, Case Western Reserve Univ School of Medicine, Cleveland, Ohio (H.A., M.O.S.); Michigan Inst for Neurologic Disorders, Farmington Hills, Mich (Y.M.D.); Inst of Clinical Neuroimmunology, LMU Hosp, Ludwig-Maximillians Universität München, Munich, Germany (J.H.); Dept of Neurology, Slagelse Hosps, Odense, Denmark (N.A.); Insts of Regional Health Research & Molecular Medicine, Univ of Southern Denmark, Odense, Denmark (N.A.); Dept of Radiology, Aleris Hosp, Copenhagen, Denmark (P.B.S.); NYU Multiple Sclerosis Comprehensive Care Center, Dept of Neurology, NYU School of Medicine, New York, NY (I.K.); Dept of Neurology, Center for Neurology and Neuropsychiatry, LVR-Klinikum, Heinrich Heine Univ Düsseldorf, Düsseldorf, Germany (M.R.); School of Medicine and Dentistry, Gold Coast Campus, Griffith Univ, Queensland, Australia (S.B., S.A.); Dept of Neurology, Gold Coast Univ Hosp, Queensland, Australia (S.A.); Dept of Pediatrics, Univ of Utah, Salt Lake City, Utah (B.M., A.M.J., M.W., S.G., L.J.C.); Dept of Medicine, Divs of Molecular Medicine & Infectious Diseases, and Ludquist Inst for Biomedical Innovation at Harbor-UCLA Medical Center, Torrance, Calif (M.R.Y.); Dept of Medicine, David Geffen School of Medicine at UCLA, Los Angeles, Calif (M.R.Y.); Depts of Ophthalmology and Visual Sciences, Kellogg Eye Center, Univ of Michigan, Ann Arbor, Mich (T.J.S.); Div of Metabolism, Endocrine and Diabetes, Dept of Internal Medicine, Univ of Michigan Medical School, Ann Arbor, Mich (T.J.S.); Hoffmann-LaRoche, Basel, Switzerland (J.W.); Dept of Neurology, Charité-Universitätsmedizin Berlin, Germany (F.P.); Affiliated author members of the Guthy-Jackson Charitable Foundation (GJCF) International Clinical Consortium (ICC) for NMOSD are listed in Appendix S1.
Abstract:
Background Patients with neuromyelitis optica spectrum disorder (NMOSD) are often seropositive for antibodies against aquaporin-4 (AQP4). The importance of MRI monitoring in this disease requires evaluation. Purpose To profile MRI features from a large international cohort with AQP4 immunoglobulin G (IgG)-seropositive NMOSD (from the Parallel MRI in NMOSD [PAMRINO] study) and to evaluate and confirm existing knowledge regarding the incidence, location, and longitudinal development of characteristic lesions in the central nervous system associated with AQP4-IgG-seropositive NMOSD. Materials and Methods In this retrospective study (from August 2016 to January 2019), MRI and clinical data were collected from 17 NMOSD expert sites in 11 countries across four continents. Clinical features and lesions identified at cross-sectional and longitudinal MRI were assessed. No formal statistical tests were used to compare observations; however, means, SDs, and 95% CIs are reported when evaluating lesion frequencies. Results Available T1-weighted and T2-weighted MRI scans in patients with AQP4-IgG-seropositive NMOSD (n = 525) were read. Among the 525 patients, 320 underwent cerebral MRI examinations with T2-weighted hyperintense cerebral (264 of 320; 82.5%), cerebellar (44 of 320; 13.8%), and brainstem (158 of 321 [49.2%], including one lesion observed at cervical spinal cord [SC] MRI) lesions. Lesions in the optic nerves, analyzed from 152 MRI examinations, were mainly found in the central (81 of 92; 88%) and posterior (79 of 92; 86%) sections (bilaterally in 39 of 92; 42%). Longitudinally extensive transverse myelitis was the predominant SC lesion pattern (upper compartment from 322 MRI examinations, 133 of 210 [63.3%]; and lower compartment from 301 MRI examinations, 149 of 212 [70.3%]). However, nonlongitudinal extensive transverse myelitis lesions were also observed frequently (105 of 210; 50.0%) in the cervical SC. Clinical data (n = 349; mean age, 44 years ± 14 [SD]; 202 female patients) and acute lesions at contrast-enhanced (CE) MRI (n = 58, performed within 30 days of the last attack) were evaluated. CE lesions were detected in the cerebrum (eight of 13; 62%), optic nerves (14 of 19; 74%), or chiasm (three of four; 75%) within 15 days of any relapse. In the upper SC (29 of 44; 66%), CE lesions were frequently observed up to 20 days after a clinical myelitis event. Conclusion A high incidence of abnormal brain MRI examinations and nonlongitudinal extensive SC lesions was found in patients in PAMRINO with AQP4-IgG-seropositive NMOSD. © RSNA, 2024 Supplemental material is available for this article.
Insights
This study reveals a high frequency of brain MRI abnormalities and non-longitudinal extensive spinal cord lesions in patients with aquaporin-4 immunoglobulin G-seropositive neuromyelitis optica spectrum disorder (NMOSD). These findings highlight the importance of MRI monitoring in NMOSD.
Area of Science:
- Neurology
- Radiology
- Immunology
Background:
- Neuromyelitis optica spectrum disorder (NMOSD) is often associated with aquaporin-4 (AQP4) antibodies.
- The role of MRI in monitoring NMOSD requires further investigation.
- Understanding characteristic MRI lesion patterns is crucial for diagnosis and management.
Purpose of the Study:
- To characterize MRI features in a large international cohort of AQP4 immunoglobulin G (IgG)-seropositive NMOSD patients.
- To evaluate the incidence, location, and longitudinal evolution of central nervous system lesions in AQP4-IgG-seropositive NMOSD.
- To confirm existing knowledge on NMOSD lesion patterns using data from the Parallel MRI in NMOSD (PAMRINO) study.
Main Methods:
- Retrospective analysis of MRI and clinical data from 17 NMOSD expert sites across 11 countries (August 2016 - January 2019).
- Assessment of clinical features and lesions on cross-sectional and longitudinal MRI scans in 525 patients with AQP4-IgG-seropositive NMOSD.
- Evaluation of contrast-enhanced (CE) MRI findings within 30 days of the last attack.
Main Results:
- High incidence of T2-weighted hyperintense lesions observed in the cerebrum (82.5%), cerebellum (13.8%), and brainstem (49.2%).
- Optic nerve lesions predominantly affected central and posterior sections (88% and 86%, respectively).
- Longitudinally extensive transverse myelitis was common in the spinal cord (63.3% upper, 70.3% lower compartment), alongside frequent non-longitudinal extensive cervical spinal cord lesions (50.0%). CE lesions were detected in the brain, optic nerves, chiasm, and spinal cord following relapses.
Conclusions:
- A significant proportion of patients with AQP4-IgG-seropositive NMOSD exhibit abnormal brain MRI findings.
- Non-longitudinal extensive spinal cord lesions are frequently observed in this patient group.
- These findings underscore the importance of comprehensive MRI assessment in managing AQP4-IgG-seropositive NMOSD.
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