Cited by 183Genetic Neurodegenerative DiseasesNeurological diseases and metabolismIron Metabolism and Disorders

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Increased Basal Ganglia Iron Levels in Huntington Disease

Bartzokis, George, Cummings, Jeffrey, Perlman, Susan, Hance, Darwood B., Mintz, Jim·1999·Archives of Neurology

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Abstract

OBJECTIVE: To quantify in vivo brain ferritin iron levels in patients with Huntington disease (HD) and normal control subjects. DESIGN AND SUBJECTS: A magnetic resonance imaging method that can quantify ferritin iron levels with specificity in vivo was employed to study 11 patients with HD and a matched group of 27 normal controls. Three basal ganglia structures (caudate, putamen, and globus pallidus) and 1 comparison region (frontal lobe white matter) were evaluated. RESULTS: Basal ganglia iron levels were significantly increased (P<.002) in patients with HD, and this increase occurred early in the disease process. This was not a generalized phenomenon, as white matter iron levels were lower in patients with HD. CONCLUSIONS: The data suggest that increased iron levels may be related to the pattern of neurotoxicity observed in HD. Reducing the oxidative stress associated with increased iron levels may offer novel ways to delay the rate of progression and possibly defer the onset of HD.

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Authors: Bartzokis, G., Cummings, J., Perlman, S., Hance, D. B., & Mintz, J.
Affiliations: Central Arkansas Veterans Healthcare System (US)
University of California, Los Angeles (US)
ORCID: Cummings, Jeffrey
Mintz, Jim
Venue: Archives of Neurology
Publisher: American Medical Association (AMA)
Published: 1999-05-01
Volume: 56
Issue: 5
Pages: 569
ISSN: 0003-9942 (ISSN-L)
DOI: 10.1001/archneur.56.5.569
OpenAlex: W2008362268
Cited by: 183
References: 49
Citations by year: 2026: 1 · 2025: 4 · 2024: 3 · 2023: 4 · 2022: 9 · 2021: 13 · 2020: 5 · 2019: 6 · 2018: 7 · 2017: 6 · 2016: 3 · 2015: 12 · 2014: 11 · 2013: 8 · 2012: 12
FWCI: 1.55
Type: journal article
Language: en
Open access: closed
Locations: Archives of Neurology (publishedVersion)
PubMed (publishedVersion)
Topics: Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
Life Sciences › Neuroscience › Neurology › Neurological diseases and metabolism
Health Sciences › Medicine › Hematology › Iron Metabolism and Disorders
Keywords: Huntington's disease, basal ganglia, brain iron, ferritin, caudate nucleus, putamen, globus pallidus, oxidative stress, neurotoxicity, disease progression
MeSH terms: Adult, Aged, Basal Ganglia, Female, Ferritins, Humans, Huntington Disease, Male, Middle Aged, Neurotoxins, Oxidative Stress, Disease Progression

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Increased Basal Ganglia Iron Levels in Huntington Disease

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