水通道蛋白-4 免疫球蛋白 G 阳性视神经脊髓炎谱系疾病的 MRI 特征：来自国际真实世界 PAMRINO 研究队列的数据分析。
Aquaporin-4 Immunoglobulin G-seropositive Neuromyelitis Optica Spectrum Disorder MRI Characteristics: Data Analysis from the International Real-World PAMRINO Study Cohort.
机构信息
From 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.
出版信息
Radiology. 2024 Nov;313(2):e233099. doi: 10.1148/radiol.233099.
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 ( = 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 ( = 349; mean age, 44 years ± 14 [SD]; 202 female patients) and acute lesions at contrast-enhanced (CE) MRI ( = 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
背景 视神经脊髓炎谱系疾病（NMOSD）患者常为水通道蛋白 4（AQP4）抗体阳性。因此需要评估 MRI 监测在该疾病中的重要性。
目的 从 AQP4 免疫球蛋白 G（IgG）阳性 NMOSD（来自 NMOSD 的平行 MRI 研究[PAMRINO]）的大型国际队列中分析 MRI 特征，并评估和确认与 AQP4-IgG 阳性 NMOSD 相关的中枢神经系统特征性病变的发生率、位置和纵向发展的现有知识。
材料与方法 在这项回顾性研究中（2016 年 8 月至 2019 年 1 月），从四大洲 11 个国家的 17 个 NMOSD 专家中心收集了 MRI 和临床数据。评估了横断面和纵向 MRI 上的临床特征和病变。未使用正式的统计检验来比较观察结果；但是，在评估病变频率时报告了平均值、标准差和 95%置信区间。
结果 对 AQP4-IgG 阳性 NMOSD 患者（=525 例）的可用 T1 加权和 T2 加权 MRI 扫描进行了阅读。在 525 例患者中，320 例行脑 MRI 检查，T2 加权高信号脑（264/320；82.5%）、小脑（44/320；13.8%）和脑干（158/321 [49.2%]，包括 1 例颈椎脊髓[SC]MRI 病变）。152 次 MRI 检查中主要观察到视神经病变，主要位于中央（81/92；88%）和后部（79/92；86%）节段（双侧 39/92；42%）。颈椎 SC 中最常见的病变模式是长节段横贯性脊髓炎（322 次 MRI 检查中有 133 次[63.3%]为上节段病变，301 次 MRI 检查中有 149 次[70.3%]为下节段病变）。然而，也经常观察到非长节段横贯性脊髓炎病变（210 次中有 105 次[50.0%]）。评估了临床数据（=349 例；平均年龄 44 岁±14 [标准差]；202 例女性患者）和急性对比增强 MRI（CE）病变（=58 例，在最后一次发作后 30 天内进行）。CE 病变在任何复发后 15 天内可在脑（8/13；62%）、视神经（14/19；74%）或视交叉（4/4；75%）中检测到。在颈椎 SC 中（44/44；66%），上 SC 的 CE 病变常在临床脊髓炎事件后 20 天内观察到。
结论 PAMRINO 中 AQP4-IgG 阳性 NMOSD 患者的脑 MRI 检查异常和非长节段广泛 SC 病变发生率较高。
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