PDF(Pubmed)
Abstract：
Superficial siderosis (SS) of the central nervous system is a rare disorder that is caused by chronic or recurrent hemorrhage in the subarachnoid space via a dural defect at the spinal level. The most common clinical features of SS include slow-progressive sensorineural deafness, cerebellar symptoms, and pyramidal tract signs. Considering that SS can present with broad clinical manifestations, for precise diagnosis, this disease must be understood. Anti-Ro/SSA antibodies are commonly detected in patients with Sjögren\'s syndrome and are utilized as markers for autoimmune diseases. In this report, we present a unique pathological condition in which SS coincided with a positive anti-Ro/SSA antibody test result. During the diagnosis of gait disturbance, an elevation in anti-Ro/SSA antibody was detected, and steroid pulse therapy was initiated as the initial treatment for autoimmune diseases. Head magnetic resonance imaging (MRI) revealed extensive hypointensity as a dark band that surrounded the intracranial basal structures and cerebellar hemispheres. Spinal MRI indicated ventral longitudinal intraspinal fluid collection extending from C7 to T5 as well as a defect in the ventral T2-3 dura mater. Intraoperative visualization revealed that the intradural venous plexus was the source of bleeding that caused the SS. To our knowledge, this report is the first to discuss the presence of anti-Ro/SSA antibodies in patients with SS. The role of anti-Ro/SSA antibodies in the pathophysiology of SS remains unclear; therefore, to confirm a possible association, further research and accumulation of cases are required.
摘要：
中枢神经系统的浅表铁质沉着症（SS）是一种罕见的疾病，由蛛网膜下腔的慢性或复发性出血通过脊髓水平的硬膜缺损引起。SS最常见的临床特征包括缓慢进行性感音神经性耳聋，小脑症状,和锥体束的迹象。考虑到SS可以表现出广泛的临床表现，为了精确诊断，必须了解这种疾病。抗Ro/SSA抗体通常在干燥综合征患者中检测到，并用作自身免疫性疾病的标志物。在这份报告中,我们提出了一种独特的病理状况，其中SS与抗Ro/SSA抗体测试结果阳性一致。在诊断步态障碍期间，检测到抗Ro/SSA抗体升高，类固醇脉冲疗法开始作为自身免疫性疾病的初始治疗。头部磁共振成像（MRI）显示广泛的低张力，是围绕颅内基底结构和小脑半球的暗带。脊柱MRI显示腹侧纵向椎管内积液从C7延伸至T5，以及腹侧T2-3硬脑膜缺损。术中可视化显示，硬膜内静脉丛是导致SS的出血源。据我们所知,本报告首次讨论SS患者中是否存在抗Ro/SSA抗体.抗Ro/SSA抗体在SS病理生理学中的作用尚不清楚；因此，为了确认可能的关联，需要进一步研究和积累案例。
