机构：[1]Department of Neurology, the Second Affiliated Hospital of GuangZhou Medical University, Guangzhou, Guangdong Province, China,[2]Institute of Neuroscience and The Second Affiliated Hospital of Guangzhou Medical University, Key Laboratory of Neurogenetics and Channelopathies of Guangdong Province and the Ministry of Education of China, Collaborative Innovation Center for Neurogenetics and Channelopathies, Guangzhou, China,[3]Department of Emergency, The Second Affiliated Hospital of GuangZhou Medical University, Guangzhou, Guangdong Province, China,[4]Department of Neurology, The Third Affiliated Hospital of Sun Yat-Sen University, Guangzhou, Guangdong Province, China,中山大学附属第三医院[5]Department of Neurology, The Affiliated Hospital of Qingdao University, Qingdao, Shandong Province, China,[6]Department of Neurology, The Fifth Affiliated Hospital of GuangZhou Medical University, Guangzhou, Guangdong Province, China,[7]Department of Neurology, The Second Chinese Medicine Hospital of Guangdong Province, Guangzhou, Guangdong Province, China,[8]Department of Neurology, Guangdong 999 Brain Hospital, Guangzhou, Guangdong Province, China,[9]Department of Neurology, Yunfu City People’s Hospital, Yunfu, Guangdong Province, China
Although rare, brain abnormalities without optic neuritis (ON) or transverse myelitis (TM) diagnosed with neuromyelitis optica spectrum disorder (NMOSD) have been reported in patients positive for the aquaporin-4 (AQP4) antibody.
To analyze demographic and clinical differences among NMOSD patients without ON or TM, those with either ON or TM, and patients with simultaneous ON and TM at disease onset.
In this retrospective study, patients who were positive for the AQP4 antibody, as detected using a cell-based assay, at the Second Affiliated Hospital of Guangzhou Medical University in China were recruited. Demographic and clinical data were obtained from each patient's medical record.
A total of 292 patients were included in this study and were divided into four subgroups based on their initial manifestations: (i) NMOSD without ON or TM (NMOSD-ON-TM-, n = 70); (ii) NMOSD with ON (NMOSD-ON+, n = 95); (iii) NMOSD with TM (NMOSD-TM+, n = 116); and (iv) simultaneous ON and TM [neuromyelitis optica (NMO), n = 11]. We found that age at onset was lower in the NMOSD-ON-TM- group than that in the other groups. The interval from the first episode to relapse was shorter in the NMOSD-ON-TM- group than that in NMOSD-TM+ group. Cerebral spinal fluid white cell counts and protein levels were significantly higher in the NMOSD-ON-TM- group than those in the other groups. Lower Expanded Disability Status Scale scores were observed in the NMOSD-ON-TM- group. Brain abnormalities, including in area postrema and hemisphere lesions, were more frequent in the NMOSD-ON-TM- group. Kaplan-Meier analysis showed that patients in the NMOSD-ON-TM- group experienced earlier relapse than those in other groups. Conversion to NMO in the NMOSD-ON+ group was greater than that in the other groups. Only 14 patients (4.8%, 14/292) had pure brain abnormalities, of which 12 had disease duration of several more years and 8 (57.1%) experienced relapses.
NMOSD patients with different initial manifestations present with significant differences in clinical features during follow-up. Patients with long-term AQP4 autoimmunity in the brain in the absence of ON or TM are not common.
基金：
This study was supported by the Natural Science Foundation
of Guangdong Province (2014A030313499) and the Science
and Technology plan project of Guangdong Province
(2014A020212332).
第一作者机构：[1]Department of Neurology, the Second Affiliated Hospital of GuangZhou Medical University, Guangzhou, Guangdong Province, China,[2]Institute of Neuroscience and The Second Affiliated Hospital of Guangzhou Medical University, Key Laboratory of Neurogenetics and Channelopathies of Guangdong Province and the Ministry of Education of China, Collaborative Innovation Center for Neurogenetics and Channelopathies, Guangzhou, China,
通讯作者：
通讯机构：[1]Department of Neurology, the Second Affiliated Hospital of GuangZhou Medical University, Guangzhou, Guangdong Province, China,[2]Institute of Neuroscience and The Second Affiliated Hospital of Guangzhou Medical University, Key Laboratory of Neurogenetics and Channelopathies of Guangdong Province and the Ministry of Education of China, Collaborative Innovation Center for Neurogenetics and Channelopathies, Guangzhou, China,
推荐引用方式(GB/T 7714)：
Youming Long,Junyan Liang,Linzhan Wu,et al.Different Phenotypes at Onset in Neuromyelitis Optica Spectrum Disorder Patients with Aquaporin-4 Autoimmunity.[J].FRONTIERS IN NEUROLOGY.2017,8:doi:10.3389/fneur.2017.00062.
APA：
Youming Long,Junyan Liang,Linzhan Wu,Shaopeng Lin,Cong Gao...&Quanxi Su.(2017).Different Phenotypes at Onset in Neuromyelitis Optica Spectrum Disorder Patients with Aquaporin-4 Autoimmunity..FRONTIERS IN NEUROLOGY,8,
MLA：
Youming Long,et al."Different Phenotypes at Onset in Neuromyelitis Optica Spectrum Disorder Patients with Aquaporin-4 Autoimmunity.".FRONTIERS IN NEUROLOGY 8.(2017)
