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Increased Basal Ganglia Iron Levels in Huntington Disease
George Bartzokis, MD;
Jeffrey Cummings, MD;
Susan Perlman, MD;
Darwood B. Hance, MD;
Jim Mintz, PhD
Arch Neurol. 1999;56:569-574.
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.
From the Department of Psychiatry, University of Arkansas for Medical Sciences (Dr Bartzokis) and the Mental Health Service, Central Arkansas Veterans Healthcare System(Dr Bartzokis), Little Rock; the Psychiatry Service, West Los Angeles Veterans Affairs Medical Center, Los Angeles, Calif (Dr Bartzokis), and the Departments of Psychiatry (Drs Bartzokis, Cummings, and Mintz), Neurology (Drs Cummings and Perlman), and Radiology (Dr Hance), University of California, Los Angeles.
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