RESUMEN
Objective To investigate the clinic characters and diagnosis of superficial siderosis in the central nervous system (SSCN).Methods One patient was systematically studied by the authors. Results SSCN was a rare entity,resulting in the deposition of ferric pigments and ions on the surface of the central nervous system.The clinical features included progressive sensorineural hearing loss,cerebellar ataxia and pyramidal sign,widespread hypointensity band at surfaces of the cerebral or cerebellar hemispheres,the brain stem and the spinal cord on Gradient Echo T_2~*-weighted images (GRE-T_2~* WI) of MR,elevation of the levels of ferritin in the cerebrospinal fluid.Conclusions This disease can be identified at early stage with history and physical examination.GRE-T_2~* WI and some related cerebrospinal fluid tests will contribute to diagnosis.