Two-year observational study of deferiprone in superficial siderosis

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Update on a patient with superficial siderosis on deferiprone.

Deferiprone Superficial siderosis (SS) is a neurodegenerative condition caused by hemosiderin deposition on the surface of the brain, cranial nerves, and spinal cord. SS is an exceptionally rare condition characterized by a triad of hearing loss, ataxia, and myelopathy. The most common etiologies that lead to SS are trauma, previous surgical procedures, dural tears, and tumors of the central ne...

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Deferiprone reduces hemosiderin deposits in the brain of a patient with superficial siderosis.

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BACKGROUND AND PURPOSE Superficial siderosis is a neurodegenerative disease caused by toxic accumulation of hemosiderin on the surface of the brain and spinal cord for which there is no known effective treatment. METHODS Oral deferiprone, a lipid-soluble iron chelator with ability to cross the blood-brain barrier, at a dose of 30 mg/kg per day was tested for safety in an open pilot study in 1...

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Superficial siderosis.

Case 1 An elderly patient presented with insidious onset of sensorineural hearing loss. The past history was unremarkable except for insidious onset of weakness of left lower limb. Physical examination revealed left hemiparesis with upgoing left plantar. Magnetic resonance imaging (MRI) of brain revealed a large flow void in the interpeduncular cistern suggestive of aneurysm arising from the to...

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ژورنال

عنوان ژورنال: CNS Neuroscience & Therapeutics

سال: 2017

ISSN: 1755-5930

DOI: 10.1111/cns.12792