Superficial siderosis of the central nervous system : MRI findings and clinical significance

We reviewed a 7-year series of brain MRI examinations to determine the frequency and clinical significance of superficial siderosis of the central nervous system (SS). SS was defined by widespread bilateral signal loss at the surfaces of the cerebral or cerebellar hemispheres, the brain stem and the spinal cord on T2-weighted images. Clinical data comprised a neurological examination of identified patients and a review of their case notes. Among 8843 consecutive studies we identified 13 (0.15%) patients with MRI evidence of SS. Only 2 had symptoms or signs characteristic of SS, such as cerebellar ataxia, hearing loss, myelopathy and dementia. Haemosiderin deposition was most widespread in both symptomatic individuals. A definite cause for SS was detected in 9 patients (69%). None of them had a full clinical picture of SS. These data indicate SS per se to be much more frequent than may be assumed from the literature. It appears to become symptomatic only with extensive amounts of widespread iron deposition which develop preferentially with cryptic or unidentified causes of bleeding.