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Superficial hemosiderosis of the central nervous system
Author(s) -
River Y.,
Honigman S.,
Gomori J. M.,
Reches A.
Publication year - 1994
Publication title -
movement disorders
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.352
H-Index - 198
eISSN - 1531-8257
pISSN - 0885-3185
DOI - 10.1002/mds.870090509
Subject(s) - superficial siderosis , hemosiderosis , medicine , siderosis , dysarthria , hemifacial spasm , chelation therapy , ferritin , ataxia , gastroenterology , magnetic resonance imaging , central nervous system disease , pathology , surgery , radiology , disease , dementia , facial nerve , alternative medicine , cerebral amyloid angiopathy , psychiatry
Abstract Superficial hemosiderosis (SH) of the CNS is a rare disease caused by repeated subarachnoid hemorrhage, with progressive superficial siderosis of the CNS. 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‐chelating 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.