Department of Neurology, Mayo Clinic, Rochester, Minnesota, USA.
Department of Radiology, Mayo Clinic, Rochester, Minnesota, USA.
J Neurol Neurosurg Psychiatry. 2017 Mar;88(3):218-225. doi: 10.1136/jnnp-2016-314738. Epub 2016 Dec 2.
We assessed the frequency and characteristics of ring-enhancing spinal cord lesions in neuromyelitis optica spectrum disorder (NMOSD) myelitis and myelitis of other cause.
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).
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).
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.
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。
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）。
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）。
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。
