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Superficial hemosiderosis of the central nervous system

1994; Wiley; Volume: 9; Issue: 5 Linguagem: Inglês

10.1002/mds.870090509

1531-8257

Yaron River, S. Honigman, John M. Gomori, Avinoam Reches,

Hemoglobinopathies and Related Disorders

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.