Two-year observational study of deferiprone in superficial siderosis.

ABSTRACT:

Introduction

Superficial siderosis is a rare, neurodegenerative disease caused by toxic accumulation of hemosiderin on the surface of the brain and the spinal cord, most commonly from chronic subarachnoid hemorrhage.

Aims

The aim of this study was to assess the clinical and radiological outcomes of superficial siderosis patients using deferiprone, a cell permeant iron chelator. Subjects obtained pre- and post-treatment brain MRIs and weekly laboratory tests. Osirix software was used to develop a method of quantifying hemosiderin deposition. Three-dimensional whole brain images of gradient echo images were rendered and compared by dividing the mean T2 hyperintensity to the maximal cerebrospinal fluid signal.

Results

A total of 38 subjects completed the study, of which

SUBMITTER: Kessler RA 

PROVIDER: S-EPMC6489821 | biostudies-literature | 2018 Mar

REPOSITORIES: biostudies-literature