Siyuan Fan1, Yan Xu2, Haitao Ren1, Hongzhi Guan1, Feng Feng3, Xuehui Gao4, Ding Ding5, Fang Fang5, Guangliang Shan6, Tianjia Guan7, Yao Zhang1, Yi Dai1, Ming Yao1, Bin Peng1, Yicheng Zhu1, Liying Cui8. 1. Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China. 2. Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China. Electronic address: xuyanpumch@hotmail.com. 3. Department of Radiology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China. 4. Department of Laboratory, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China. 5. Department of Neurology, Beijing Children's Hospital, Capital Medical University, National Center for Children's Health, China. 6. Department of Epidemiology and Statistics, Institute of Basic Medical Sciences, Chinese Academy of Medical Sciences, Beijing, China; School of Basic Medicine, Peking Union Medical College, Beijing, China. 7. School of Public Health, Peking Union Medical College, Beijing, China. 8. Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China; Neurosciences Center, Chinese Academy of Medical Sciences, Beijing, China.
Abstract
BACKGROUND: Myelin oligodendrocyte glycoprotein (MOG)-antibody (ab) disease and AQP4-IgG-positive neuromyelitis optica spectrum disorder (NMOSD) can co-exist with anti-NMDA (N-methyl-D-aspartate) receptor encephalitis (NMDARe). OBJECTIVES: To characterize MOG-ab disease and AQP4-IgG-positive NMOSD during NMDARe. METHODS: We analyzed all the patients with overlapping MOG-ab disease and NMDARe (MNOS) and patients with AQP4-IgG-positive NMOSD and NMDARe (ANOS) in our hospital and compared those data with data from systematically review of previously published reports. RESULTS: In our cohorts, 11.9% patients with MOG-ab disease and 0.6% patients with NMOSD had overlapping NMDARe (P < 0.01). After treatment with steroids and/or intravenous immunoglobulin (IVIg), the median modified Rankin Scale (mRS) of the MNOS group decreased significantly during attacks associated with or without NMDARe (P < 0.01 for both), while that of the ANOS group did not (attack: P < 0.05; attack associated with NMDARe: P > 0.05). Analyzed together with previously reported cases, 6% patients with MNOS and 40% patients with ANOS also used rituximab or cyclophosphamide after steroids and/or IVIg (P < 0.05) during attacks associated with NMDARe. CONCLUSION: Compared with NMOSD, MOG-ab disease may more commonly co-exist with NMDARe. MNOS patients respond better to steroids and IVIg than do ANOS patients during attacks associated with NMDARe.
BACKGROUND:Myelin oligodendrocyte glycoprotein (MOG)-antibody (ab) disease and AQP4-IgG-positive neuromyelitis optica spectrum disorder (NMOSD) can co-exist with anti-NMDA (N-methyl-D-aspartate) receptor encephalitis (NMDARe). OBJECTIVES: To characterize MOG-ab disease and AQP4-IgG-positive NMOSD during NMDARe. METHODS: We analyzed all the patients with overlapping MOG-ab disease and NMDARe (MNOS) and patients with AQP4-IgG-positive NMOSD and NMDARe (ANOS) in our hospital and compared those data with data from systematically review of previously published reports. RESULTS: In our cohorts, 11.9% patients with MOG-ab disease and 0.6% patients with NMOSD had overlapping NMDARe (P < 0.01). After treatment with steroids and/or intravenous immunoglobulin (IVIg), the median modified Rankin Scale (mRS) of the MNOS group decreased significantly during attacks associated with or without NMDARe (P < 0.01 for both), while that of the ANOS group did not (attack: P < 0.05; attack associated with NMDARe: P > 0.05). Analyzed together with previously reported cases, 6% patients with MNOS and 40% patients with ANOS also used rituximab or cyclophosphamide after steroids and/or IVIg (P < 0.05) during attacks associated with NMDARe. CONCLUSION: Compared with NMOSD, MOG-ab disease may more commonly co-exist with NMDARe. MNOS patients respond better to steroids and IVIg than do ANOS patients during attacks associated with NMDARe.
Authors: S Jarius; F Paul; O Aktas; N Asgari; R C Dale; J de Seze; D Franciotta; K Fujihara; A Jacob; H J Kim; I Kleiter; T Kümpfel; M Levy; J Palace; K Ruprecht; A Saiz; C Trebst; B G Weinshenker; B Wildemann Journal: Nervenarzt Date: 2018-12 Impact factor: 1.214
Authors: S Jarius; F Paul; O Aktas; N Asgari; R C Dale; J de Seze; D Franciotta; K Fujihara; A Jacob; H J Kim; I Kleiter; T Kümpfel; M Levy; J Palace; K Ruprecht; A Saiz; C Trebst; B G Weinshenker; B Wildemann Journal: J Neuroinflammation Date: 2018-05-03 Impact factor: 8.322