[Superficial cerebral hemosiderosis: a rare cause of ataxia or hypoacusis]

Abstract

Superficial siderosis of the central nervous system (SSC) typically involves slowly progressive ataxia, hypoacusis and dementia, possibly with pyramidal signs and sphincter disturbances in combination with xanthochromic CSF with siderophages. However, there are also atypical oligosymptomatic forms. Before the era of magnetic resonance imaging (MRI), diagnosis was only possible at autopsy. Nowadays a firm diagnosis can be made during life by demonstrating the typical marginal hypointense signal in the T2-weighted images in the cerebrum, brain stem, cerebellum and spinal cord. Contrast-enhanced computed tomography may demonstrate widespread meningeal enhancement, but this sign is not specific for SSC. We present an oligosymptomatic case of SSC with slowly progressive ataxia and slight hypoacusis. The etiology, gross pathological and histopathological findings, differential diagnosis and therapy are discussed.