Agranulocytosis with deferiprone treatment of superficial siderosis. (7th August 2013)

• Record Type: Journal Article
• Authors: Huprikar, Nikhil; Gossweiler, Marisa; Callaghan, Maureen; Bunge, Paul
• Is Part Of: BMJ case reports. Volume 2013
• Publication Date: 2013-08-07
• Subjects: Medicine -- Case studies -- Periodicals; 610.5
• DOI: 10.1136/bcr-2013-010099
• Languages: English
• ISSNs: 1757-790X

Abstract

Abstract : Superficial siderosis of the central nervous system is a rare neurological disorder caused by deposits of haemosiderin on subplial brain matter. Characterised by a thin dark layer surrounding the brain stem, cerebellum and cortical fissures on the T2-weighted MRI, symptoms include sensorineural hearing loss and progressive gait ataxia. A specific aetiology for the blood in the subarachnoid space is identified in less than 50% of cases. While identification of a specific vascular defect allows for vascular repair, treatment options are limited for idiopathic superficial siderosis. Recently, a pilot safety study demonstrated promising results using an iron chelator, deferiprone. While this approach is promising, we present a potential serious complication of this therapy—the first report of agranulocytosis in the treatment of superficial siderosis following deferiprone therapy.