Brief Communication

Use of desferrioxamine in the treatment of aceruloplasminemia

  1. Hiroaki Miyajima MD1,
  2. Yoshitomo Takahashi MD1,
  3. Tadashi Kamata MD1,
  4. Hideaki Shimizu MD1,
  5. Naoki Sakai MD1,
  6. Dr. Jonathan D. Gitlin MD2,*

Article first published online: 8 OCT 2004

DOI: 10.1002/ana.410410318

Issue

Annals of Neurology

Annals of Neurology

Volume 41, Issue 3, pages 404–407, March 1997

Additional Information

How to Cite

Miyajima, H., Takahashi, Y., Kamata, T., Shimizu, H., Sakai, N. and Gitlin, J. D. (1997), Use of desferrioxamine in the treatment of aceruloplasminemia. Ann Neurol., 41: 404–407. doi: 10.1002/ana.410410318

Author Information

  1. 1

    First Department of Medicine, Hamamatsu University School of Medicine, Hamamatsu, Japan

  2. 2

    Edward Mallinckrodt Department of Pediatrics, Washington University School of Medicine, St. Louis, MO

*Washington University School of Medicine, Department of Pediatrics, St. Louis Children's Hospital, One Children's Place, St. Louis, MO 63110

Publication History

  1. Issue published online: 8 OCT 2004
  2. Article first published online: 8 OCT 2004
  3. Manuscript Revised: 5 SEP 1996
  4. Manuscript Accepted: 5 SEP 1996
  5. Manuscript Received: 17 JUN 1996

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Abstract

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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