title: Secondary intracranial hypertension with acute intracranial pressure crisis in superficial siderosis.
abstract: 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.
authors.affiliation: Department of Neurology and Clinical Neurophysiology, Academic Hospital Bogenhausen, Technical University of Munich, Englschalkingerstr. 77 D-81925 Munich, Germany. steve_linder@gmx.de
authors.forename: Stefan
authors.initials: S
authors.lastname: Linder
authors.forename: Dennis A
authors.initials: DA
authors.lastname: Nowak
authors.forename: Sven-Olaf
authors.initials: SO
authors.lastname: Rodiek
authors.forename: Christianto
authors.initials: C
authors.lastname: Lumenta
authors.forename: Helge
authors.initials: H
authors.lastname: Topka
mesh_terms: D000328:Adult; D002493:Central Nervous System Diseases / Q000209:etiology* / Q000473:pathology; D006485:Hemosiderin / Q000009:adverse effects; D006801:Humans; D019586:Intracranial Hypertension / Q000209:etiology* / Q000628:therapy; D008279:Magnetic Resonance Imaging; D008297:Male; D012806:Siderosis / Q000150:complications* / Q000473:pathology; D016896:Treatment Outcome; D017287:Ventriculoperitoneal Shunt
chemical_list: D006485:Hemosiderin