Mao Zhifeng, Yin Junjie, Zhong Xiaonan, Zhao Zhihua, Qiu Wei, Lu Zhengqi, Hu Xueqiang
Multiple Sclerosis Center, Department of Neurology, The Third Affiliated Hospital of Sun Yat-Sen University, 600 Tianhe Road, Guangzhou, Guangdong, 510630, China.
BMC Neurol. 2015 Sep 4;15:160. doi: 10.1186/s12883-015-0417-y.
Increasing 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.
This 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.
We 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.
This 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.
据报道，晚发型患者（晚发型视神经脊髓炎谱系障碍，LONMOSD）中抗水通道蛋白4（AQP4）血清阳性的视神经脊髓炎谱系障碍（NMOSD）发病率呈上升趋势。然而，早发型和晚发型变体之间临床差异的全貌仍不清楚。我们描述了中国人群中抗AQP4血清阳性的LONMOSD患者的临床特征和转归。
这是一项回顾性病历分析，该队列研究纳入了2006年1月至2014年2月期间抗AQP4血清阳性的早发型（≤49岁）和晚发型（≥50岁）NMOSD患者。分析了人口统计学、临床、神经影像学和脑脊液（CSF）检查结果以及预后数据。
我们确定了30例抗AQP4血清阳性的LONMOSD患者（86.7%为女性）。发病的中位年龄为57.5岁（范围50 - 70岁）。视神经炎（ON）和长节段横贯性脊髓炎（LETM）的发病频率相似。（首次）ON与LETM之间的间隔时间更长（中位时间13个月对4个月；p < 0.05），从首次症状到诊断为NMO的时间更长（中位时间17个月对7个月，p < 0.05），合并症更多（66.7%对26.7%；p < 0.05），高血压更多见（26.7%对3.3%；p < 0.05）。NMO样病变较少见（10.7%对41.6%；p < 0.05），而非特异性病变的发生率往往更高（53.6%对29%；p = 0.067）。这些患者在病情最低点时的扩展残疾状态量表（EDSS）评分更严重（中位评分6.75对5；p < 0.05）。发作常在短时间内导致EDSS达到4级（中位时间8个月对13.5个月；p < 0.05）。在最后一次随访时，这些患者的EDSS评分更严重（中位评分5.25对4；p < 0.05）。未发现显著的预测因素。
本研究概述了中国抗AQP4血清阳性的LONMOSD患者的临床和副临床特征，并显示了早发型与晚发型之间的一些独特疾病特征。老年患者在病程较短时更容易出现残疾。然而，这些患者在脑部并不总是表现出NMO样病变。与之前的报道相反，初始LETM不一定是主要的首发症状。较高的合并症可能需要调整治疗方法。
