Late-onset neuromyelitis optica spectrum disorder in AQP4-seropositivepatients in a Chinese population.
Late-onset neuromyelitis optica spectrum disorder in AQP4-seropositivepatients in a Chinese population.
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中国人群中 AQP4 血清阳性患者的迟发性视神经脊髓炎谱系障碍
DOI：
10.1186/s12883-015-0417-y
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发表时间：
2015-09-04
期刊：
影响因子：
2.6
通讯作者：
Hu X
中科院分区：
文献类型：
--
作者：
Mao Z;Yin J;Zhong X;Zhao Z;Qiu W;Lu Z;Hu X
研究背景：在晚发性视神经肌萎缩症（LONMOSD）患者中，AQP 4血清阳性的视神经肌萎缩症谱系障碍（NMOSD）的发病率增加。然而，早发性和晚发性变异体之间的全部临床差异仍不清楚。我们描述了AQP 4血清阳性LONMOSD患者在中国population.MethodsThis的临床特征和结果是一个回顾性分析的病历队列研究AQP 4血清阳性NMOSD患者的早发性（≤49岁）和晚发性（≥50岁）之间的变异2006年1月和2014年2月。人口统计学，临床，神经影像学和脑脊液（CSF）的结果和预后data.ResultsWe确定了30 AQP 4血清阳性LONMOSD患者（86.7%的妇女）进行了分析。中位发病年龄为57.5岁（范围50-70岁）。视神经炎（ON）与纵向广泛性横肌炎（LETM）的发病率相似。（首次）ON和LETM之间的间隔较长（中位13 vs. 4个月; p 0.05），从首次症状到诊断出NMO的时间较长（中位17 vs. 7个月，p 0.05），合并症较多（66.7 vs. 26.7%; p 0.05），高血压较多（26.7 vs.3.3%; p 0.05）。NMO样病变较不常见（10.7 vs. 41.6%; p 0.05），而非特异性病变的发生率往往较高（53.6 vs. 29%; p = 0.067）。这些患者的扩展残疾状态量表（EDSS）评分最低时更严重（中位数6.75 vs.5; p 0.05）。发作通常在短时间内导致EDSS 4（中位8个月vs. 13.5个月; p 0.05）。末次随访时，这些患者的EDSS评分更严重（中位数5.25 vs. 4; p 0.05）。没有显着的predictors were identified.ConclusionsThis研究提供了一个概述的AQP 4-血清阳性LONMOSD患者在中国的临床和临床旁特征，并证明了一些不同的疾病特征，在早期与晚发型。老年患者在短期内更容易出现残疾。然而，这些患者并不总是在大脑中显示出NMO样病变。与以前的报告相反，最初的LETM可能不一定是主要的初始症状。较高的合并症可能需要修改治疗方法。
BackgroundIncreasing rates of AQP4-seropositive neuromyelitis optica spectrum disorder (NMOSD) have been reported in late-onset patients (LONMOSD). However, the full range of clinical differences between early-onset and late-onset variants remain unclear. We describe the clinical features and outcomes of AQP4-seropositive LONMOSD patients in a Chinese population.MethodsThis was a retrospective analysis of medical records in a cohort study of AQP4-seropositive NMOSD patients with early-onset (≤49 years) and late-onset (≥50 years) variants between January 2006 and February 2014. Demographic, clinical, neuroimaging and cerebrospinal fluid (CSF) findings and prognosis data were analyzed.ResultsWe identified thirty AQP4-seropositive LONMOSD patients (86.7 % women). The median age at onset was 57.5 years (range 50–70). There were similar onset frequencies between optic neuritis (ON) and longitudinally extensive transverse myelitis (LETM). Longer interval between (first) ON and LETM (median 13 vs. 4 months; p < 0.05), time from first symptoms to diagnosis of NMO (median 17 vs. 7 months, p < 0.05), higher comorbidities (66.7 vs. 26.7 %; p < 0.05), and more hypertension (26.7 vs.3.3 %; p < 0.05) were prevalent. NMO-like lesions were less common (10.7 vs. 41.6 %; p < 0.05), while the rate of non-specific lesions tended to be higher (53.6 vs. 29 %; p = 0.067). These patients displayed more severe Expanded Disability Status Scale (EDSS) in nadir (median 6.75vs.5; p < 0.05). Attacks often resulted in EDSS 4 within a short period (median 8 vs. 13.5 months; p < 0.05). At last follow-up, the EDSS score was more severe in these patients (median 5.25 vs. 4; p < 0.05). No significant predictors were identified.ConclusionsThis study provides an overview of the clinical and paraclinical features of AQP4-seropositive LONMOSD patients in China and demonstrates a number of distinct disease characteristics in early vs. late onset. Older patients are more susceptible to disability in short course. However, these patients do not always display NMO-like lesions in the brain. Initial LETM may not necessarily be predominant as the initial symptom, contrary to previous reports. The higher comorbidities may warrant a modified approach of treatment.