Long Youming, Liang Junyan, Wu Linzhan, Lin Shaopeng, Gao Cong, Chen Xiaohui, Qiu Wei, Yang Yu, Zheng Xueping, Yang Ning, Gao Min, Chen Yaotang, Wang Zhanhang, Su Quanxi
Department of Neurology, the Second Affiliated Hospital of GuangZhou Medical University, Guangzhou, Guangdong Province, China; 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.
Department of Emergency, The Second Affiliated Hospital of GuangZhou Medical University , Guangzhou, Guangdong Province , China.
Front Neurol. 2017 Feb 28;8:62. doi: 10.3389/fneur.2017.00062. eCollection 2017.
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-ONTM, = 70); (ii) NMOSD with ON (NMOSD-ON, = 95); (iii) NMOSD with TM (NMOSD-TM, = 116); and (iv) simultaneous ON and TM [neuromyelitis optica (NMO), = 11]. We found that age at onset was lower in the NMOSD-ONTM group than that in the other groups. The interval from the first episode to relapse was shorter in the NMOSD-ONTM group than that in NMOSD-TM group. Cerebral spinal fluid white cell counts and protein levels were significantly higher in the NMOSD-ONTM group than those in the other groups. Lower Expanded Disability Status Scale scores were observed in the NMOSD-ONTM group. Brain abnormalities, including in area postrema and hemisphere lesions, were more frequent in the NMOSD-ONTM group. Kaplan-Meier analysis showed that patients in the NMOSD-ONTM 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.
尽管罕见，但已报道水通道蛋白4（AQP4）抗体阳性的视神经脊髓炎谱系障碍（NMOSD）患者存在无视神经炎（ON）或横贯性脊髓炎（TM）的脑异常情况。
分析疾病发作时无ON或TM的NMOSD患者、有ON或TM的NMOSD患者以及同时患有ON和TM的患者在人口统计学和临床方面的差异。
在这项回顾性研究中，招募了在中国广州医科大学附属第二医院通过基于细胞的检测方法检测出AQP4抗体阳性的患者。从每位患者的病历中获取人口统计学和临床数据。
本研究共纳入292例患者，并根据其初始表现分为四个亚组：（i）无ON或TM的NMOSD（NMOSD-ONTM，n = 70）；（ii）有ON的NMOSD（NMOSD-ON，n = 95）；（iii）有TM的NMOSD（NMOSD-TM，n = 116）；以及（iv）同时患有ON和TM [视神经脊髓炎（NMO），n = 11]。我们发现，NMOSD-ONTM组的发病年龄低于其他组。NMOSD-ONTM组从首次发作到复发的间隔时间比NMOSD-TM组短。NMOSD-ONTM组的脑脊液白细胞计数和蛋白水平显著高于其他组。NMOSD-ONTM组的扩展残疾状态量表得分较低。NMOSD-ONTM组脑异常情况更常见，包括最后区和半球病变。Kaplan-Meier分析表明，NMOSD-ONTM组患者比其他组患者复发更早。NMOSD-ON组向NMO的转化高于其他组。只有14例患者（4.8%，14/292）有单纯脑异常，其中12例病程长达数年，8例（57.1%）经历复发。
具有不同初始表现的NMOSD患者在随访期间的临床特征存在显著差异。在无ON或TM的情况下，长期存在脑内AQP4自身免疫的患者并不常见。
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