{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T10:33:12Z","timestamp":1790764392764,"version":"4.1.0"},"reference-count":15,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2017,12,28]],"date-time":"2017-12-28T00:00:00Z","timestamp":1514419200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["CNS Neurosci Ther"],"published-print":{"date-parts":[[2018,3]]},"abstract":"<jats:title>Summary<\/jats:title><jats:sec><jats:title>Introduction<\/jats:title><jats:p>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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Aims<\/jats:title><jats:p>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\u2010 and post\u2010treatment brain <jats:styled-content style=\"fixed-case\">MRI<\/jats:styled-content>s and weekly laboratory tests. Osirix software was used to develop a method of quantifying hemosiderin deposition. Three\u2010dimensional whole brain images of gradient echo images were rendered and compared by dividing the mean T2 hyperintensity to the maximal cerebrospinal fluid signal.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A total of 38 subjects completed the study, of which clinical and radiological data were available for 30. The average age was 64\u00a0years (range 37\u201086), 53% were male, 94% were white. Nineteen subjects (63%) reported either no progression of disease or an improvement in at least one neurological domain, with 40% of patients reporting a stabilization in hearing function and 30% reporting stable or improved coordination and walking. By <jats:styled-content style=\"fixed-case\">MRI<\/jats:styled-content>, there was an overall mean increase in T2 hyperintensity of the whole brain of 1%\u201013% over the 2\u2010year time period in half of patients, indicating a reduction hemosiderosis. There were no cases of agranulocytosis, and declines of white blood cells counts and neutrophils averaged &lt;10%. Fatigue was the most common side effect.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>This is the first long\u2010term prospective study of superficial siderosis on the iron chelator, deferiprone. <jats:styled-content style=\"fixed-case\">MRI<\/jats:styled-content> quantification of hemosiderin appears to demonstrate a measurable reduction in half of patients and this correlated with a stabilized or improving disease course. A future placebo\u2010controlled trial is necessary to determine whether deferiprone is an effective therapy for superficial siderosis.<\/jats:p><\/jats:sec>","DOI":"10.1111\/cns.12792","type":"journal-article","created":{"date-parts":[[2017,12,29]],"date-time":"2017-12-29T04:32:43Z","timestamp":1514521963000},"page":"187-192","source":"Crossref","is-referenced-by-count":44,"title":["Two\u2010year observational study of deferiprone in superficial siderosis"],"prefix":"10.1111","volume":"24","author":[{"given":"Remi A.","family":"Kessler","sequence":"first","affiliation":[{"name":"Department of Neurology Johns Hopkins University  Baltimore MD USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xu","family":"Li","sequence":"additional","affiliation":[{"name":"FM Kirby Research Center for Functional Brain Imaging Kennedy Krieger Institute  Baltimore MD USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kateryna","family":"Schwartz","sequence":"additional","affiliation":[{"name":"Department of Neurology Johns Hopkins University  Baltimore MD USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hwa","family":"Huang","sequence":"additional","affiliation":[{"name":"Department of Neurology Johns Hopkins University  Baltimore MD USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maureen A.","family":"Mealy","sequence":"additional","affiliation":[{"name":"Department of Neurology Johns Hopkins University  Baltimore MD USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7969-8346","authenticated-orcid":false,"given":"Michael","family":"Levy","sequence":"additional","affiliation":[{"name":"Department of Neurology Johns Hopkins University  Baltimore MD USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2017,12,28]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/ncpneuro0356"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000208510.76323.5b"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.410340505"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.24850"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1136\/bcr-2013-009916"},{"key":"e_1_2_7_7_1","doi-asserted-by":"crossref","first-page":"219","DOI":"10.1017\/cjn.2016.329","article-title":"Deferiprone reduces hemosiderin deposition in superficial siderosis","volume":"44","author":"Kuo PH","year":"2017","journal-title":"Can J Neurol Sci"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.3174\/ajnr.A2331"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.3174\/ajnr.A3186"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1097\/00006123-199712000-00028"},{"key":"e_1_2_7_11_1","doi-asserted-by":"publisher","DOI":"10.1161\/STROKEAHA.111.628032"},{"key":"e_1_2_7_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.spinee.2009.03.004"},{"key":"e_1_2_7_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajh.24479"},{"key":"e_1_2_7_14_1","doi-asserted-by":"publisher","DOI":"10.1111\/ejh.12241"},{"key":"e_1_2_7_15_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1749-6632.1998.tb10478.x"},{"key":"e_1_2_7_16_1","doi-asserted-by":"publisher","DOI":"10.2214\/AJR.09.7215"}],"container-title":["CNS Neuroscience &amp; Therapeutics"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fcns.12792","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/cns.12792","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,26]],"date-time":"2023-09-26T16:32:47Z","timestamp":1695745967000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/cns.12792"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,12,28]]},"references-count":15,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2018,3]]}},"alternative-id":["10.1111\/cns.12792"],"URL":"https:\/\/doi.org\/10.1111\/cns.12792","archive":["Portico"],"relation":{},"ISSN":["1755-5930","1755-5949"],"issn-type":[{"value":"1755-5930","type":"print"},{"value":"1755-5949","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,12,28]]}}}