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水通道蛋白 4 抗体阳性的长节段横贯性脊髓炎的 MRI 特征:对视神经脊髓炎谱系疾病诊断的启示。

MRI Features of Aquaporin-4 Antibody-Positive Longitudinally Extensive Transverse Myelitis: Insights into the Diagnosis of Neuromyelitis Optica Spectrum Disorders.

机构信息

From the Departments of Radiology (C.G.C., J.W.L., H.W.A., E.L., Y.K., H.S.K.).

Neurology (K.S.P.), Seoul National University Bundang Hospital, Gyeonggi-do, Korea.

出版信息

AJNR Am J Neuroradiol. 2018 Apr;39(4):782-787. doi: 10.3174/ajnr.A5551. Epub 2018 Feb 15.

Abstract

BACKGROUND AND PURPOSE

Longitudinally extensive transverse myelitis is a well-documented spinal manifestation of neuromyelitis optica spectrum disorders, however, other forms of nontumorous myelopathy can also manifest as longitudinally extensive transverse myelitis. Our aim was to evaluate the MR imaging features of aquaporin-4 antibody-positive longitudinally extensive transverse myelitis, which is strongly associated with neuromyelitis optica spectrum disorders.

MATERIALS AND METHODS

We evaluated cervicomedullary junction involvement, cord expansion ratios, bright spotty lesions, the number of involved segments, skipped lesions, enhancement patterns, and axial distribution patterns using spinal MR imaging of 41 patients with longitudinally extensive transverse myelitis who underwent aquaporin-4 antibody testing. Univariate logistic regression analysis was performed to identify factors associated with aquaporin-4 antibody seropositivity, which were then used to develop a scoring system for diagnosing aquaporin-4 antibody-positive longitudinally extensive transverse myelitis. Interrater reliability for cord expansion ratio measurement and bright spotty lesions was determined using intraclass correlation coefficients and κ values, respectively.

RESULTS

Fifteen patients with longitudinally extensive transverse myelitis were aquaporin-4 antibody-positive. Sex (female), cervicomedullary junction involvement, a cord expansion ratio of >1.4, and bright spotty lesions were significantly associated with aquaporin-4 antibody seropositivity. The sensitivity and specificity of the scoring system were 73.3% and 96.2%, respectively. The interclass correlation value for the cord expansion ratio was 0.78, and the κ value for bright spotty lesions was 0.61.

CONCLUSIONS

Our scoring system, based on cervicomedullary junction involvement, higher cord expansion ratio, bright spotty lesions, and female sex, can facilitate the timely diagnosis of neuromyelitis optica spectrum disorders.

摘要

背景与目的

长节段横贯性脊髓炎是视神经脊髓炎谱系疾病的一种典型脊髓表现,然而,其他形式的非肿瘤性脊髓病也可能表现为长节段横贯性脊髓炎。我们的目的是评估水通道蛋白 4 抗体阳性的长节段横贯性脊髓炎的磁共振成像(MR)特征,该疾病与视神经脊髓炎谱系疾病密切相关。

材料与方法

我们评估了 41 例接受水通道蛋白 4 抗体检测的长节段横贯性脊髓炎患者的颈髓交界处受累、脊髓扩张比、斑片状高信号病灶、受累节段数、跳跃性病灶、强化模式和轴向分布模式。采用单因素逻辑回归分析确定与水通道蛋白 4 抗体血清阳性相关的因素,并据此建立一个诊断水通道蛋白 4 抗体阳性的长节段横贯性脊髓炎的评分系统。采用组内相关系数和κ 值分别评估脊髓扩张比和斑片状高信号病灶的测量者间信度。

结果

15 例长节段横贯性脊髓炎患者的水通道蛋白 4 抗体阳性。女性、颈髓交界处受累、脊髓扩张比>1.4 和斑片状高信号病灶与水通道蛋白 4 抗体血清阳性显著相关。评分系统的敏感性和特异性分别为 73.3%和 96.2%。脊髓扩张比的组内相关系数为 0.78,斑片状高信号病灶的κ 值为 0.61。

结论

我们的评分系统基于颈髓交界处受累、更高的脊髓扩张比、斑片状高信号病灶和女性,有助于及时诊断视神经脊髓炎谱系疾病。

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