The clinical and radiological features of superficial siderosis of central nervous system: report of four cases

Objective To explore the etiology, clinical features and treatment of superficial siderosis of central nervous system (SSCNS) in China. Methods The clinical data of four patients with SSCNS diagnosed by magnetic resonance imaging (MRI) and susceptibility weighted imaging (SWI) from Qilu Hospital of Shandong University during 2015—2019 were retrospectively reviewed. The etiology and clinical features of the four cases were summarized and analyzed. Results All the four patients were male, with an average age of 60.5 years. Clinical symptoms included headache, hearing loss, and cerebellar ataxia. Brain MRI and SWI showed that hemosiderin mainly deposited (short linear T2 signal, low SWI signal) on the surface of cerebellum, brainstem, temporal lobe, frontal lobe, and spinal cord. Potential bleeding sites were found in all four patients, including brain traumatic history, spinal intradural extramedullary cavernous hemangioma, brain metastasis and intracranial aneurysm. Patients were followed up for six months to four years. Headache symptoms improved in only one patient who received surgical treatment, while symptoms of the other three patients progressed. Conclusions SSCNS is mainly characterized by hearing loss, progressive cerebellar ataxia and myelopathy. The diagnosis of this disease mainly depends on imaging examination. The linear low signal on the surface of T2WI is the main basis for the diagnosis of SSCNS. Surgical treatment of bleeding sites and iron chelator are the main treatments of the disease. Key words: Hemosiderosis; Central nervous system diseases; Magnetic resonance imaging