Superficial siderosis of central nervous system with cerebrospinal fluid oligoclonal bands: a case report and expert comments

DING Jie; LIN Zhi; CHEN Ying; XU Qun; LI Jianping; CHEN Zengai; GUAN Yangtai

doi:10.12022/jnnr.2017-0074

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Journal of Neurology and Neurorehabilitation ›› 2017, Vol. 13 ›› Issue (4) : 180-185. DOI: 10.12022/jnnr.2017-0074

Superficial siderosis of central nervous system with cerebrospinal fluid oligoclonal bands: a case report and expert comments

DING Jie1, LIN Zhi1, CHEN Ying1, XU Qun1, LI Jianping1, CHEN Zengai2, GUAN Yangtai1

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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.

Key words

Superficial siderosis of central nervous system / Oligoclonal bands / Neuromyelitis optica spectrum disorders

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DING Jie, LIN Zhi, CHEN Ying, XU Qun, LI Jianping, CHEN Zengai, GUAN Yangtai. Superficial siderosis of central nervous system with cerebrospinal fluid oligoclonal bands: a case report and expert comments[J]. Journal of Neurology and Neurorehabilitation. 2017, 13(4): 180-185 https://doi.org/10.12022/jnnr.2017-0074

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