Michishita Yuko, Saito Takuya, Uchiyama Tsuyoshi
Department of Neurology, Seirei Hamamatsu General Hospital, Hamamatsu, Japan.
Radiol Case Rep. 2024 Jan 13;19(4):1276-1279. doi: 10.1016/j.radcr.2024.01.006. eCollection 2024 Apr.
Sturge-Weber syndrome (SWS) is a rare congenital disorder associated with systemic vascular malformations characterized by port-wine stains, epilepsy, and glaucoma. Patients with SWS can develop stroke-like symptoms such as hemiparesis. We report a case of a 63-year old woman with SWS who developed left-sided hemiparesis and was finally diagnosed with myelin-oligodendrocyte glycoprotein (MOG) antibody-positive encephalitis. Brain magnetic resonance imaging (MRI) revealed right-dominant bilateral leptomeningeal enhancement, thickened dura mater, and a cerebellar lesion. Cerebrospinal fluid (CSF) examination showed pleocytosis. Both serum and CSF proved positive for MOG antibodies. The patient recovered immediately after intravenous methylprednisolone administration. SWS and MOG antibody-positive encephalitis share similar clinical findings of stroke-like symptoms and leptomeningeal enhancement on MRI. However, MOG antibody-positive encephalitis is highly steroid-responsive in most cases. If a patient with SWS develops stroke-like symptoms accompanied by abnormal CSF findings or subtentorial lesions, testing for MOG antibodies should be considered.
斯特奇-韦伯综合征（SWS）是一种罕见的先天性疾病，与系统性血管畸形相关，其特征为葡萄酒色斑、癫痫和青光眼。SWS患者可出现类似中风的症状，如偏瘫。我们报告一例63岁患有SWS的女性，她出现左侧偏瘫，最终被诊断为髓鞘少突胶质细胞糖蛋白（MOG）抗体阳性脑炎。脑磁共振成像（MRI）显示右侧为主的双侧软脑膜强化、硬脑膜增厚以及小脑病变。脑脊液（CSF）检查显示细胞增多。血清和脑脊液的MOG抗体均呈阳性。患者静脉注射甲泼尼龙后立即康复。SWS和MOG抗体阳性脑炎在MRI上具有类似中风症状和软脑膜强化的相似临床表现。然而，MOG抗体阳性脑炎在大多数情况下对类固醇高度敏感。如果SWS患者出现类似中风的症状并伴有脑脊液异常或幕下病变，应考虑检测MOG抗体。
