视神经脊髓炎谱系疾病中的环形强化脊髓病变。
Ring-enhancing spinal cord lesions in neuromyelitis optica spectrum disorders.
机构信息
Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
Department of Radiology, Mayo Clinic, Rochester, Minnesota, USA.
出版信息
J Neurol Neurosurg Psychiatry. 2017 Mar;88(3):218-225. doi: 10.1136/jnnp-2016-314738. Epub 2016 Dec 2.
OBJECTIVE
We assessed the frequency and characteristics of ring-enhancing spinal cord lesions in neuromyelitis optica spectrum disorder (NMOSD) myelitis and myelitis of other cause.
METHODS
We reviewed spinal cord MRIs for ring-enhancing lesions from 284 aquaporin-4 (AQP4)-IgG seropositive patients at Mayo Clinic from 1996 to 2014. Inclusion criteria were as follows: (1) AQP4-IgG seropositivity, (2) myelitis attack and (3) MRI spinal cord demonstrating ring-enhancement. We identified two groups of control patients with: (1) longitudinally extensive myelopathy of other cause (n=66) and (2) myelitis in the context of a concurrent or subsequent diagnosis of multiple sclerosis (MS) from a population-based cohort (n=30).
RESULTS
Ring-enhancement was detected in 50 of 156 (32%) myelitis episodes in 41 patients (83% single; 17% multiple attacks). Ring-enhancement was noted on sagittal and axial images in 36 of 43 (84%) ring enhancing myelitis episodes and extended a median of two vertebral segments (range, 1-12); in 21 of 48 (44%) ring enhancing myelitis episodes, the ring extended greater than or equal to three vertebrae. Ring-enhancement was accompanied by longitudinally extensive (greater than or equal to three vertebral segments) T2-hyperintensity in 44 of 50 (88%) ring enhancing myelitis episodes. One case of a spinal cord biopsy during ring-enhancing myelitis revealed tissue vacuolation and loss of AQP4 immunoreactivity with preserved axons. The clinical characteristics of ring-enhancing myelitis episodes did not differ from non-ring-enhancing episodes. Ring-enhancing spinal cord lesions were more common in NMOSD than other causes of longitudinally extensive myelopathy (50/156 (32%) vs 0/66 (0%); p≤0.001) but did not differ between NMOSD and MS (50/156 (32%) vs 6/30 (20%); p=0.20).
CONCLUSIONS
Spinal cord ring-enhancement accompanies one-third of NMOSD myelitis episodes and distinguishes NMOSD from other causes of longitudinally extensive myelopathies but not from MS.
目的
我们评估视神经脊髓炎谱系疾病（NMOSD）性脊髓炎和其他原因性脊髓炎中环形增强脊髓病变的频率和特征。
方法
我们回顾了 1996 年至 2014 年期间在 Mayo 诊所的 284 名水通道蛋白 4（AQP4）-IgG 阳性患者的脊髓 MRI 中环形增强病变。纳入标准如下：（1）AQP4-IgG 阳性，（2）脊髓炎发作，（3）MRI 显示脊髓环形增强。我们从基于人群的队列中确定了两组对照患者：（1）其他原因引起的长节段性脊髓病（n=66）和（2）多发性硬化症（MS）并发或随后诊断为脊髓炎（n=30）。
结果
在 41 名患者（83%为单发；17%为多发）的 156 次（32%）脊髓炎发作中，发现 50 次（32%）环形增强。在 36 次（84%）环形增强性脊髓炎发作中，在矢状位和轴位图像上均可见环形增强，并延伸了中位数两个椎体节段（范围，1-12）；在 48 次（44%）环形增强性脊髓炎发作中，环形增强大于或等于三个椎体。在 50 次（88%）环形增强性脊髓炎发作中，环形增强伴有大于或等于三个椎体节段的纵向广泛（大于或等于三个椎体节段）T2 高信号。在 50 次（32%）环形增强性脊髓炎发作中，1 例脊髓活检显示组织空泡化和 AQP4 免疫反应性丧失，伴有保留的轴突。环形增强性脊髓炎发作的临床特征与非环形增强性发作无差异。环形增强性脊髓病变在 NMOSD 中比其他长节段性脊髓病（50/156（32%）与 0/66（0%）；p≤0.001）更为常见，但在 NMOSD 和 MS 之间无差异（50/156（32%）与 6/30（20%）；p=0.20）。
结论
脊髓环形增强伴随三分之一的 NMOSD 性脊髓炎发作，并将 NMOSD 与其他长节段性脊髓病区分开来，但不能与 MS 区分开来。
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