Weibi Chen1, Qian Li2, Ting Wang3, Linlin Fan1, Lehong Gao1, Zhaoyang Huang1, Yicong Lin1, Qin Xue1, Gang Liu1, Yingying Su1, Yan Zhang4. 1. Department of Neurology, Xuanwu Hospital Capital Medical University, China. 2. Department of Neurology, Xuanwu Hospital Capital Medical University, China; Department of Neurology, Haihe Clinical College of Tianjin Medical University, China. 3. Department of Neurology, Xuanwu Hospital Capital Medical University, China; Department of Neurology, Songyuan Central Hospital, China. 4. Department of Neurology, Xuanwu Hospital Capital Medical University, China. Electronic address: zhangyanlq@xwhosp.org.
Abstract
BACKGROUND: The co-existence of anti-N-methyl-D-aspartate receptor encephalitis (NMDARe) and anti-myelin oligodendrocyte glycoprotein (MOG) antibody disease has sparsely been reported, which needs to be investigated. METHOD: Among the patients with NMDARe in Xuanwu Hospital, MOG antibody disease and NMDARe overlapping syndrome (MNOS) were retrospectively identified. We combined our data with those from previously reported cases to characterize this new entity. RESULT: There were 45 patients with MNOS with a median onset age of 20. A total of 97.8% of the patients had symptoms of encephalitis; 68.9% of the patients had symptoms of demyelination, including optic neuritis (ON) (37.9%), longitudinally extensive transverse myelitis (LETM) (31.0%) and acute disseminated encephalomyelitis (ADEM) (27.6%). Abnormal signals on magnetic resonance imaging (MRI) usually involved cortical (46.7%), subcortical (31.1%) and basal ganglia (26.7%) lesions, as well as infratentorial (48.9%) and spinal cord (28.9%) lesions. No tumours were found. A total of 62.2% of the patients relapsed, with recurrence rates of 66.7% and 50.0% for those treated with first-line therapy alone and in combination with second-line immunotherapy, respectively. The pathological changes from the biopsy indicated immune-mediated inflammatory demyelination. Although some patients may have residual deficits, 93.3% of the patients became functionally independent. CONCLUSION: The possibility of MNOS should be considered when patients diagnosed with anti-NMDARe simultaneously or sequentially develop ON, LETM or ADEM. MNOS occurred without tumour association, and inflammatory demyelination may be the pathological change. Steroids combined with second-line immunotherapy can help to reduce high recurrence rates, and most patients will have substantial recovery.
BACKGROUND: The co-existence of anti-N-methyl-D-aspartate receptor encephalitis (NMDARe) and anti-myelin oligodendrocyte glycoprotein (MOG) antibody disease has sparsely been reported, which needs to be investigated. METHOD: Among the patients with NMDARe in Xuanwu Hospital, MOG antibody disease and NMDARe overlapping syndrome (MNOS) were retrospectively identified. We combined our data with those from previously reported cases to characterize this new entity. RESULT: There were 45 patients with MNOS with a median onset age of 20. A total of 97.8% of the patients had symptoms of encephalitis; 68.9% of the patients had symptoms of demyelination, including optic neuritis (ON) (37.9%), longitudinally extensive transverse myelitis (LETM) (31.0%) and acute disseminated encephalomyelitis (ADEM) (27.6%). Abnormal signals on magnetic resonance imaging (MRI) usually involved cortical (46.7%), subcortical (31.1%) and basal ganglia (26.7%) lesions, as well as infratentorial (48.9%) and spinal cord (28.9%) lesions. No tumours were found. A total of 62.2% of the patients relapsed, with recurrence rates of 66.7% and 50.0% for those treated with first-line therapy alone and in combination with second-line immunotherapy, respectively. The pathological changes from the biopsy indicated immune-mediated inflammatory demyelination. Although some patients may have residual deficits, 93.3% of the patients became functionally independent. CONCLUSION: The possibility of MNOS should be considered when patients diagnosed with anti-NMDARe simultaneously or sequentially develop ON, LETM or ADEM. MNOS occurred without tumour association, and inflammatory demyelination may be the pathological change. Steroids combined with second-line immunotherapy can help to reduce high recurrence rates, and most patients will have substantial recovery.