Clinical, radiographic characteristics and immunomodulating changes in neuromyelitis optica with extensive brain lesions.
Clinical, radiographic characteristics and immunomodulating changes in neuromyelitis optica with extensive brain lesions.
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DOI：
10.1186/1471-2377-13-72
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发表时间：
2013-07-03
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：
Cheng C;Jiang Y;Chen X;Dai Y;Kang Z;Lu Z;Peng F;Hu X
视神经肌萎缩症（NMO）表现为各种脑磁共振成像（MRI）异常，伴有反复发作的中枢神经系统（CNS）发作，但主要影响脊髓和视神经。然而，NMO与大脑的广泛参与很少被研究。我们调查了中国NMO患者的临床、影像学特征和免疫调节变化。对2006年1月至2010年2月期间住院的NMO患者（包括16例伴有EBL的NMO患者和53例不伴有EBL的NMO患者）进行回顾性分析。分析两组患者的临床特征、MRI表现、实验室检查、治疗情况及转归。所有患者均接受了2年的随访。根据其MRI特征将NMO的EBL分为4类：1）肿瘤样病变（n=4，25%）; 2）急性播散性脑脊髓炎（ADEM）样病变多发性硬化（MS）样病变（n = 5，31.25%）;后部可逆性脑病综合征（PRES）样病变（n=1，6.25%）。与无EBL的NMO患者（NEBL）相比，有EBL的NMO患者的脑病症状（37.5% vs. 5.6%，p = 0.004）、同侧偏盲（18.8% vs. 0%，p = 0.011）和AQP 4血清阳性（100% vs. 69.8%，p = 0.008）发生率更高。EBL组C3、C4、ESR、CRP水平明显高于非EBL组。随访期间EBL的复发次数比NEBL更频繁（1.88 ± 0.30 vs. 1.23 ± 0.14，p = 0.04）。在整个随访期间，EBL患者的EDSS评分也明显高于NEBL患者。NMO中EBL的存在可能预示着疾病的活动性增高，预后不良。CRP是监测疾病活动性的有用指标。全身性炎症可能对NMO中EBL的形成至关重要。
Neuromyelitis optica (NMO) shows various brain magnetic resonance imaging (MRI) abnormalities with recurrent central nervous system (CNS) attacks, although predominantly affecting the spinal cord and optic nerve. However, NMO with extensive involvement of the brain has infrequently been studied. We investigated the clinical, radiographic features and immunomodulating changes of NMO patients with extensive brain lesions (EBLs) in China. NMO patients (including 16 NMO patients with EBLs and 53 NMO patients without EBLs) hospitalized during January 2006 and February 2010 were recruited and analyzed retrospectively. Data of clinical characteristics, magnetic resonance imaging (MRI) features, laboratory abnormalities, treatment details and outcomes were analyzed. All the patients received the follow-up visits for two years. EBLs in NMO were classified into four categories according to their respective MRI characteristics: 1) Tumefactive-like lesions (n=4, 25%); 2) Acute disseminated encephalomyelitis (ADEM)-like lesions (n=6, 37.5%); 3) Multiple sclerosis (MS)-like lesions (n=5, 31.25%); 4) Posterior reversible encephalopathy syndrome (PRES)-like lesions (n=1, 6.25%). NMO patients with EBLs had higher rates of encephalopathy symptoms (37.5% vs. 5.6%, p = 0.004), homonymous hemianopia (18.8% vs. 0%, p = 0.011) and AQP4 seropositivity (100% vs. 69.8%, p = 0.008) than NMO patients without EBLs (NEBLs). Immunomodulating changes (including the levels of C3, C4, ESR and CRP) were significantly higher in patients with EBLs than those without EBLs. The relapse times in EBLs during the follow-up period were more frequent than those happened in NEBLs (1.88 ± 0.30 vs. 1.23 ± 0.14, p = 0.04). The EDSS scores in EBLs patients were also much higher than those in NEBLs throughout all the whole visits of follow-up. The presence of EBLs in NMO may indicate a higher diseases activity and portend a worse prognosis. CRP is a useful marker in monitoring diseases activity. Systemic inflammation may be crucial to the formation of EBLs in NMO.
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