Department of Neurology, Korea University Anam Hospital, Seoul, South Korea.
Department of Neurology, Seoul National University College of Medicine, Seoul, South Korea.
Cerebellum. 2019 Jun;18(3):511-518. doi: 10.1007/s12311-019-01018-4.
This study aimed to define the clinical features and involved structures that aid in differentiation of neuromyelitis optica spectrum disorder (NMOSD) from multiple sclerosis (MS) involving the brainstem and cerebellum. We analyzed the clinical and ocular motor findings, and lesion distribution on brain MRIs in 42 patients with MS (17 men, mean age ± SD = 37 ± 12) and 26 with NMOSD (3 men, mean age ± SD = 43 ± 15) that were recruited from two university hospitals in South Korea (whole study population). An additional subgroup analysis was also conducted in 41 patients presenting acute brainstem or vestibular syndrome (brainstem syndrome population). Logistic regression analysis showed that bilaterality of the lesions (p = 0.012) and presence of horizontal gaze-evoked nystagmus (hGEN, p = 0.041) were more frequently associated with NMOSD than with MS in the whole study population. In the brainstem syndrome population, only hGEN (p = 0.017) was more frequent in NMOSD than in MS. The lesions specific for NMOSD were overlapped in the medial vestibular nucleus (MVN) and nucleus prepositus hypoglossi (NPH) at the pontomedullary junction. In conclusion, presence of hGEN and bilateral lesions involving the MVN and NPH favor the diagnosis of NMOSD rather than MS.
本研究旨在定义有助于鉴别视神经脊髓炎谱系疾病（NMOSD）与累及脑干和小脑的多发性硬化（MS）的临床特征和受累结构。我们分析了韩国两所大学医院招募的 42 例 MS 患者（17 名男性，平均年龄 ± 标准差=37 ± 12 岁）和 26 例 NMOSD 患者（3 名男性，平均年龄 ± 标准差=43 ± 15 岁）的临床和眼动发现以及脑 MRI 上的病变分布（全研究人群）。还对 41 例表现为急性脑干或前庭综合征（脑干综合征人群）的患者进行了亚组分析。Logistic 回归分析显示，在全研究人群中，病变的双侧性（p=0.012）和水平扫视诱发的眼球震颤（hGEN，p=0.041）的存在与 NMOSD 比 MS 更相关。在脑干综合征人群中，只有 hGEN（p=0.017）在 NMOSD 中比在 MS 中更常见。NMOSD 特有的病变重叠于桥延髓交界的内侧前庭核（MVN）和舌下神经前置核（NPH）。总之，hGEN 的存在和累及 MVN 和 NPH 的双侧病变有助于诊断 NMOSD 而不是 MS。
