SUPERFICIAL SIDEROSIS OF THE CENTRAL-NERVOUS-SYSTEM - A RARE CAUSE OF ATAXIA AND HYPACUSIS

Superficial siderosis of the central nervous system (SSC) typically involves slowly progressive ataxia, hypacusis 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 hypacusis. The etiology, gross pathological and histopathological findings, differential diagnosis and therapy are discussed.