Secondary intracranial hypertension with acute intracranial pressure crisis in superficial siderosis

Superficial siderosis of the central nervous system is a very rare disease related to hemosiderin deposits in the brain, brainstem, cerebellum and spinal cord due to chronic subarachnoid hemorrhage. Chronic increased intracranial pressure develops in about one-third of affected cases. We report a patient with superficial siderosis and sudden intracranial pressure crisis. A 29-year-old man experienced a subacute episode of headache, tinnitus and blurred vision. Magnetic resonance imaging of the brain revealed hemosiderin deposits characteristic of superficial siderosis. Extensive diagnostic work-up excluded causative pathologies of bleeding. Lumbar puncture and continuous intra-ventricular cerebrospinal fluid (CSF) pressure monitoring revealed continuous CSF pressure increase. Implantation of a ventriculo-peritoneal shunt led to complete clinical recovery. Our case emphasizes that patients with superficial siderosis may present with sudden elevation of intracranial pressure due to chronic intracranial hypertension. In this situation permanent CSF drainage provides a useful therapeutic option.