PDF(Pubmed)
Abstract：
Primary central nervous system vasculitis (PCNSV) is an angiitis localized to the central nervous system (CNS), with various manifestations and no specific biomarkers. Herein, we report a case of PCNSV that presented with an unusual course. A 40-year-old Japanese male developed inner ear symptoms and visual field disturbances. Later, at 42 years of age, the patient developed right hemiparesis and was diagnosed with multiple sclerosis (MS). He received methylprednisolone pulse therapy, which improved his symptoms and resolved most brain lesions. Subsequently, he did not visit the hospital for 13 years, during which time he experienced no relapse. At 55 years of age, he presented to our hospital with fatigue and dizziness. Susac syndrome was suspected because of sensorineural hearing loss and snowball lesions in the corpus callosum. Some of the brain lesions resolved spontaneously. A biopsy was performed on a right frontal lobe lesion, which revealed vasculitis with fibrinoid necrosis, no demyelinating lesions, no amyloid positivity, and no infiltration of atypical lymphocytes. With no evidence of vasculitis in other organs, the patient was diagnosed with PCNSV. The patient was treated with methylprednisolone pulse therapy, followed by oral prednisolone (1 mg/kg/day). The prednisolone was tapered off, and no relapse of symptoms or new lesions on magnetic resonance imaging (MRI) were noted. As observed in this case, even in a scenario suggestive of Susac syndrome or multiple sclerosis, PCNSV should be considered a differential diagnosis and confirmed via brain biopsy.
摘要：
原发性中枢神经系统血管炎（PCNSV）是一种位于中枢神经系统（CNS）的血管炎，有各种表现，没有特异性的生物标志物。在这里，我们报告了一例PCNSV，表现出一个不寻常的过程。一名40岁的日本男性出现内耳症状和视野障碍。稍后，在42岁时，患者出现右侧偏瘫,并被诊断为多发性硬化(MS).他接受了甲基强的松龙脉冲治疗，改善了他的症状并解决了大部分脑部病变.随后，他已经13年没有去医院了，在此期间，他没有复发。55岁时，他带着疲劳和头晕来到我们医院。怀疑Susac综合征是因为感觉神经性听力丧失和call体的雪球病变。一些脑部病变自发消退。对右额叶病变进行了活检，显示血管炎伴有纤维蛋白样坏死，无脱髓鞘病变,没有淀粉样蛋白阳性,无非典型淋巴细胞浸润。没有其他器官血管炎的证据，患者被诊断为PCNSV.患者接受甲基强的松龙脉冲治疗，然后口服泼尼松龙（1mg/kg/天）。泼尼松龙逐渐变细，并且在磁共振成像(MRI)上没有发现症状复发或新的病变.正如在这种情况下观察到的，即使在Susac综合征或多发性硬化症的情况下，PCNSV应被视为鉴别诊断，并通过脑活检证实。
