Clinical characteristics, course and prognosis of relapsing Devic's Neuromyelitis Optica
Clinical characteristics, course and prognosis of relapsing Devic's Neuromyelitis Optica
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DOI：
10.1007/s00415-004-0271-0
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发表时间：
2004-01-01
影响因子：
6
通讯作者：
Comi, G
中科院分区：
文献类型：
--
作者：
Ghezzi, A;Bergamaschi, R;Comi, G
目的 评估 Devic 视神经脊髓炎 (DNO) 的临床特征、病程和预后，评估人口统计学和临床特征的预后作用，评估当前的 DNO 诊断标准。方法 从 15 个意大利 MS 中心收集受 DNO 影响的患者的人口统计学、临床、脑脊液和 MRI 数据。纳入标准为： 1) 两次或两次以上急性神经功能障碍发作，表明视神经和脊髓受累，同时或相继出现时间关系； 2）没有证据表明病变超出视神经或脊髓； 3) 发病时脑部 MRI 呈阴性或对多发性硬化症 (MS) 非特异性（白质病变：！2）。残疾程度通过库茨克扩展残疾状况量表（EDSS）进行评分。结果 纳入 46 例复发性 DNO 患者，其中女性 37 例，男性 9 例，平均发病年龄 40.1 +/- 16.3 岁（范围 12-77 岁）。随访时间为 8.8 +/- 3.5 年，平均年复发率为 1.3 +/- 1.2。 5、10和15年后，分别有65%、82%和86%的病例达到EDSS 3.0，分别有42%、53%和69%的病例达到EDSS 6.0，分别有8%、12%和23%的病例达到EDSS 10。达到 EDSS 3 级的概率与发病年龄、第一次和第二次发作之间的间隔以及复发率具有统计相关性。达到 EDSS 6.0 的概率与发病时的残余 EDSS 和复发率相关。随访期间，8名受试者出现脑白质病变。脊髓 MRI 显示 39 名受试者中病变延伸至 3 个或更多节段，4 名受试者中仅 1 名病变涉及 1 个节段，3 名受试者正常。 29/44 名患者（65.9%）至少发现一次或多种脑脊液异常，最常见的发现是细胞增多（38.6%）、寡克隆带（34.1%）、高蛋白水平（25%）和高白蛋白比率（20.5%）。结论 大多数情况下 DNO 预后较差。与MS相比，DNO患者发病年龄较高，女性多见，病程较严重。脑和脊髓 MRI 可将 DNO 与 MS 区分开来。如果 CSF 显示细胞和蛋白质增加，则支持 DNO 的可能性。
Objectives To evaluate the clinical characteristics, course and prognosis of Devic's neuromyelitis optica (DNO), to evaluate the prognostic role of demographic and clinical features, to evaluate the current DNO diagnostic criteria. Methods Demographic, clinical, CSF and MRI data of patients affected by DNO were collected from fifteen Italian MS centres. Inclusion criteria were: 1) two or more acute episodes of neurological dysfunction indicating involvement of the optic nerve and spinal cord, in a simultaneous or subsequent temporal relationship; 2) no evidence of lesions beyond the optic nerve or the spinal cord; 3) brain MRI at onset negative or non-specific for multiple sclerosis (MS) (white matter lesions :! 2). Disability was scored by means of Kurtzke's Expanded Disability Status Scale (EDSS). Results 46 patients with relapsing DNO were included, 37 females and 9 males, with mean age at onset of 40.1 +/- 16.3 years (range 12-77 years). The follow up duration was 8.8 +/- 3.5 years, the mean annualised relapse rate was 1.3 +/- 1.2. After 5, 10 and 15 years EDSS 3.0 was reached respectively by 65%, 82% and 86% of cases, EDSS 6.0 respectively by 42%, 53% and 69% of cases, EDSS 10 respectively by 8%, 12% and 23% of cases. The probability of reaching EDSS 3 was statistically correlated with age at onset, interval between the first and 2(nd) attack, and relapse rate. The probability of reaching EDSS 6.0 was correlated with the residual EDSS at onset and to relapse rate. During the follow up, brain white matter lesions appeared in 8 subjects. Spinal cord MRI showed lesions extending across 3 or more segments in 39 subjects, only 1 lesion involving 1 segment in 4 subjects, and was normal in 3 subjects. One or more CSF abnormalities were found at least once in 29/44 patients (65.9%), the most frequent findings being pleocytosis (38.6%), oligoclonal bands (34.1%), high protein level (25%), and high albumin ratio (20.5%). Conclusions DNO has a poor prognosis in most cases. Compared with MS, DNO patients have a higher age at onset, females are more frequently affected, the course is more severe. Brain and spinal cord MRI permit the differentiation of DNO from MS. CSF supports the probability of DNO if it shows increased cells and proteins.