Increased basal ganglia iron levels in Huntington disease

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 pati ents 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 in creased (P < .002) in patients with HD, and this increase occurred early in the disease proce ss. 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 rat e of progression and possibly defer the onset of HD.