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视神经脊髓炎谱系疾病的脑和脊髓影像学特征。

Brain and cord imaging features in neuromyelitis optica spectrum disorders.

机构信息

Neuroimaging Research Unit, Institute of Experimental Neurology, Division of Neuroscience, San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy.

Department of Neurology, Institute of Experimental Neurology, Division of Neuroscience, San Raffaele Scientific Institute, Vita-Salute San Raffaele University, Milan, Italy.

出版信息

Ann Neurol. 2019 Mar;85(3):371-384. doi: 10.1002/ana.25411. Epub 2019 Jan 28.

DOI:10.1002/ana.25411
PMID:30635936
Abstract

OBJECTIVES

To validate imaging features able to discriminate neuromyelitis optica spectrum disorders from multiple sclerosis with conventional magnetic resonance imaging (MRI).

METHODS

In this cross-sectional study, brain and spinal cord scans were evaluated from 116 neuromyelitis optica spectrum disorder patients (98 seropositive and 18 seronegative) in chronic disease phase and 65 age-, sex-, and disease duration-matched multiple sclerosis patients. To identify independent predictors of neuromyelitis optica diagnosis, after assessing the prevalence of typical/atypical findings, the original cohort was 2:1 randomized in a training sample (where a multivariate logistic regression analysis was run) and a validation sample (where the performance of the selected variables was tested and validated).

RESULTS

Typical brain lesions occurred in 50.9% of neuromyelitis optica patients (18.1% brainstem periventricular/periaqueductal, 32.7% periependymal along lateral ventricles, 3.4% large hemispheric, 6.0% diencephalic, 4.3% corticospinal tract), 72.2% had spinal cord lesions (46.3% long transverse myelitis, 36.1% short transverse myelitis), 37.1% satisfied 2010 McDonald criteria, and none had cortical lesions. Fulfillment of at least 2 of 5 of absence of juxtacortical/cortical lesions, absence of periventricular lesions, absence of Dawson fingers, presence of long transverse myelitis, and presence of periependymal lesions along lateral ventricles discriminated neuromyelitis optica patients in both training (sensitivity = 0.92, 95% confidence interval [CI] = 0.84-0.97; specificity = 0.91, 95% CI = 0.78-0.97) and validation samples (sensitivity = 0.82, 95% CI = 0.66-0.92; specificity = 0.91, 95% CI = 0.71-0.99). MRI findings and criteria performance were similar irrespective of serostatus.

INTERPRETATION

Although up to 50% of neuromyelitis optica patients have no typical lesions and a relatively high percentage of them satisfy multiple sclerosis criteria, several easily applicable imaging features can help to distinguish neuromyelitis optica from multiple sclerosis. ANN NEUROL 2019;85:371-384.

摘要

目的

利用常规磁共振成像(MRI)验证能够鉴别视神经脊髓炎谱系疾病与多发性硬化的影像学特征。

方法

在这项横断面研究中,评估了 116 例处于慢性疾病期的视神经脊髓炎谱系疾病患者(98 例血清阳性,18 例血清阴性)和 65 例年龄、性别和疾病持续时间匹配的多发性硬化症患者的脑和脊髓扫描结果。为了确定视神经脊髓炎诊断的独立预测因素,在评估典型/非典型表现的患病率后,原始队列按 2:1 随机分为训练样本(进行多元逻辑回归分析)和验证样本(测试和验证所选变量的性能)。

结果

典型脑病变发生在 50.9%的视神经脊髓炎患者中(18.1%脑干脑室周围/导水管周围,32.7%侧脑室周围,3.4%大脑半球大,6.0%间脑,4.3%皮质脊髓束),72.2%有脊髓病变(46.3%长横贯性脊髓炎,36.1%短横贯性脊髓炎),37.1%符合 2010 年 McDonald 标准,无皮质病变。至少满足 5 项标准中的 2 项(无皮质下/皮质病变、无脑室周围病变、无 Dawson 指、存在长横贯性脊髓炎和侧脑室周围存在室管膜下病变)可区分训练组和验证组中的视神经脊髓炎患者(训练组:敏感性=0.92,95%置信区间[CI]:0.84-0.97;特异性=0.91,95%CI:0.78-0.97)和验证组(敏感性=0.82,95%CI:0.66-0.92;特异性=0.91,95%CI:0.71-0.99)。MRI 表现和标准的性能与血清状态无关。

结论

尽管多达 50%的视神经脊髓炎患者没有典型病变,且相当一部分患者符合多发性硬化症标准,但一些易于应用的影像学特征有助于将视神经脊髓炎与多发性硬化症区分开来。

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