目的：探讨中枢神经系统表面铁沉积症（superficial siderosis of central nervous system，SSCNS）的临床和影像学特征，以期提高临床对SSCNS的认识。
方法：报道1例SSCNS患者的临床诊治经过，邀请神经内科及影像科专家结合相关文献对SSCNS的临床和影像学特点进行解读。
结果：1例SSCNS患者的临床表现为进行性加重的小脑性共济失调、锥体束征、感觉异常和排尿障碍。脑脊液中可见深染的免疫球蛋白G（immunoglobulin G，IgG）寡克隆区带。磁共振成像显示脑干、小脑蚓部和脊髓表面铁沉积。给予去铁酮片治疗后，患者症状趋于稳定。
结论：SSCNS的临床表现复杂多样，影像学特征和脑脊液特征有助于SSCNS的早期诊断，而早期诊断与治疗有助于阻止病情的进一步发展。此外，脑脊液寡克隆区带阳性提示潜在的免疫炎性因素可能参与SSCNS的发病，其具体机制有待进一步研究。
Abstract
Objective: To investigate the clinical and imaging characteristics of superficial siderosis of central nervous system (SSCNS) and improve the understanding of SSCNS in the clinical practice.
Methods: The clinical diagnosis and treatment of a patient with SSCNS were reported. Neurology and imaging experts were invited to discuss the clinical and imaging characteristics of SSCNS based on literature review.
Results: The patient presented with progressively evolving gait ataxia, pyramidal dysfunction, sensory abnormalities and sphincter dysfunction. Cerebrospinal fluid (CSF) examination showed immunoglobulin G (IgG) oligoclonal bands. Magnetic resonance imaging (MRI) revealed hemosiderin deposition on surface of brainstem, cerebellar vermis and spinal cord. The symptoms were controlled with treatment of deferiprone.
Conclusion: The clinical manifestations of SSCNS are complex and diverse. The imaging and CSF characteristics contribute to the early diagnosis of SSCNS. Early diagnosis and treatment can help prevent further development of the disease. In addition, the demonstration of CSF-oligoclonal bands implies that inflammation might be involved in the pathogenesis of SSCNS. The mechanism remains to be confirmed by further studies.
关键词
中枢神经系统表面铁沉积症 /
寡克隆区带 /
视神经脊髓炎谱系疾病
Key words
Superficial siderosis of central nervous system /
Oligoclonal bands /
Neuromyelitis optica spectrum disorders
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参考文献
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