1043-3155 Pediatr Neurol Briefs pedneurbriefs Pediatr Neurol Briefs Pediatric Neurology Briefs Pediatr Neurol Briefs 2166-6482 1043-3155 2166-3155 Pediatric Neurology Briefs Publishers Chicago, IL, USA PNB-13-60-a 10.15844/pedneurbriefs-13-8-5 Heredo-Degenerative Diseases Neurology Pediatrics Nervous System Diseases Child Development Brain Diseases Neurosurgery Child Infant Iron Storage in Friedreich’s Ataxia http://orcid.org/0000-0002-0173-7931 Millichap J. Gordon MD 1 2 * Division of Neurology, Children's Memorial Hospital, Chicago, IL Departments of Pediatrics and Neurology, Northwestern University Feinberg School of Medicine, Chicago, IL Correspondence: Dr. J. Gordon Millichap, E-mail: jgmillichap@northwestern.edu 08 1999 01 05 2016 13 8 60 60 Copyright: © 1999 The Author(s) 1999 This work is licensed under the Creative Commons Attribution 4.0 International License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Increased iron in the dentate nucleus of patients with Friedrich’s ataxia

To test the hypothesis that iron is increased in the cerebellum of patients with Friedreich’s ataxia (FA), a multigradient echo magnetic resonance sequence for the three-dimensional imaging of brain iron-induced contrast was used in 12 patients and 23 normal subjects examined at the National Institutes of Health, Bethesda, MD.

 Friedreich’s Ataxia Multigradient Echo Magnetic Relaxation Rate

To test the hypothesis that iron is increased in the cerebellum of patients with Friedreich’s ataxia (FA), a multigradient echo magnetic resonance sequence for the three-dimensional imaging of brain iron-induced contrast was used in 12 patients and 23 normal subjects examined at the National Institutes of Health, Bethesda, MD. Relaxation rate (R2) values, the inverse of T2, in the unaffected globus pallidus were equal in FA patients and controls, but R2 values in the dentate nucleus of FA patients were significantly higher. These R2 values reflect an increased iron concentration in the dentate, which supports the hypothesis of oxidative damage as the mechanism for FA. [1]

COMMENT. Increased iron in the dentate nucleus of patients with Friedreich’s ataxia, demonstrated by magnetic resonance, points to oxidative damage as the pathogenesis.

Waldvogel D van Gelderen P Hallett M Increased iron in the dentate nucleus of patients with Friedrich’s ataxia Ann Neurol 1999 Jul 46 1 123 5 10.1002/1531-8249(199907)46:1<123::AID-ANA19>3.0.CO;2-H 10401790