Use of desferrioxamine in the treatment of aceruloplasminemia
Aceruloplasminemia is a newly recognized autosomal recessive disorder of iron metabolism resulting in neurodegeneration of the retina and basal ganglia. We report here on the treatment of a patient who developed progressive extrapyramidal symptoms that included blepharospasm, grimacing, and rigidity...
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Published in | Annals of neurology Vol. 41; no. 3; p. 404 |
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Main Authors | , , , , , |
Format | Journal Article |
Language | English |
Published |
United States
01.03.1997
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Subjects | |
Online Access | Get more information |
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Summary: | Aceruloplasminemia is a newly recognized autosomal recessive disorder of iron metabolism resulting in neurodegeneration of the retina and basal ganglia. We report here on the treatment of a patient who developed progressive extrapyramidal symptoms that included blepharospasm, grimacing, and rigidity associated with increased iron deposition in the brain and visceral organs. Treatment for 10 months with the iron chelator desferrioxamine decreased brain iron stores, prevented progression of the neurological symptoms, and reduced plasma lipid peroxidation. These data suggest that early treatment with this chelator may be useful in such patients to diminish central nervous system iron accumulation and to prevent or ameliorate neurological symptoms associated with neurodegeneration. |
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ISSN: | 0364-5134 1531-8249 |
DOI: | 10.1002/ana.410410318 |