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We report a patient with SH whose clinical picture was marked by progressive gait ataxia, hearing loss, dysarthria, and recurrent episodes of hemifacial spasm. Iron and ferritin levels in the CSF were significantly higher than in a control group of patients. Six month's treatment with the iron\u2010chelating agent trientine dihydrochloride led to clinical improvement, with a concomitant reduction of CSF iron level. We suggest that, in addition to magnetic resonance imaging findings, CSF levels of iron and ferritin should be used as diagnostic criteria for SH, as well as to estimate the efficacy of iron chelation treatment.<\/jats:p>","DOI":"10.1002\/mds.870090509","type":"journal-article","created":{"date-parts":[[2004,12,31]],"date-time":"2004-12-31T11:37:28Z","timestamp":1104493048000},"page":"559-562","source":"Crossref","is-referenced-by-count":43,"title":["Superficial hemosiderosis of the central nervous system"],"prefix":"10.1002","volume":"9","author":[{"given":"Y.","family":"River","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S.","family":"Honigman","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J. 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