Abstract
BACKGROUND: Superficial siderosis of the central nervous system (SSCNS) is a rare neurodegenerative disorder resulting from chronic hemorrhage into the subarachnoid space. This study aims to summarize its clinical and paraclinical features to improve clinical recognition. METHODS: We retrospectively analyzed the medical records of 14 patients diagnosed with SSCNS at the Affiliated Hospital of Xuzhou Medical University and Xuanwu Hospital between August 2011 and April 2023. Data on clinical manifestations, cerebrospinal fluid (CSF), imaging findings, treatment, and prognosis were collected and summarized. RESULTS: Among 14 patients (6 males, 8 females; mean age 53.1 years), the most common clinical manifestations were hearing loss (14/14, 100%), ataxia (12/14, 85.7%), and pyramidal tract dysfunction (12/14, 85.7%). Brain MRI revealed characteristic superficial siderosis in all cases, with susceptibility-weighted imaging (SWI) demonstrating superior sensitivity. Spinal cord involvement was observed in 13 patients (92.9%). CSF pressure predominantly falling within the normal range, while lumbar CSF exhibited signs of hemorrhage or yellowing, elevated red blood cell counts, and varying degrees of increased protein content. Sensorineural hearing loss was confirmed in 7/8 tested patients (87.5%). Potential bleeding sources were identified in 12 patients (85.7%), including prior surgery, trauma, and vascular abnormalities. Treatment was primarily symptomatic, with most patients (12/14) showing minimal improvement in core symptoms during hospitalization. CONCLUSION: Superficial siderosis of the central nervous system is a progressive neurodegenerative disorder characterized by the clinical triad of hearing loss, ataxia, and pyramidal tract dysfunction. MRI, particularly T2-weighted imaging (T2WI) and SWI sequences demonstrating a characteristic peripheral low-signal band, provides high diagnostic sensitivity and represents the cornerstone of diagnosis. Early identification remains challenging yet critical, while management should focus on locating the underlying bleeding source and providing comprehensive supportive care. TRIAL REGISTRATION: Not applicable.