Systemic sclerosis and superficial siderosis of the central nervous system: casuality or causality?

We describe the case of a 59 year old man with systemic sclerosis and superficial siderosis of the central nervous system characterized by progressive worsening of walking with signs of pyramidal liberation associated with cerebellar ataxia and bilateral hypoacusia. Ferritin and oligoclonal bands were found on lumbar puncture and Magnetic Resonance Imaging of the brain and spinal cord revealed a rim of hypointensity in T2-weighted images enveloping the surface of brain and spinal cord, typical of hemosiderosis. Neurological complications, both central and peripheral, are thought to be rare and coincidental in systemic sclerosis; however over the past decade, some authors have reported a high incidence (up to 40%) of neurological involvement in the disease. In this report we consider the hypothesis that superficial siderosis may be a direct complication of systemic sclerosis. To our knowledge, this case represents the first description of superficial siderosis in a patient with systemic sclerosis. © 2008 Springer-Verlag.