E08Iron Content in Brain Increases with Progression of Huntington's Disease: Longitudinal Study

• Published in: Journal of neurology, neurosurgery and psychiatry Vol. 85; no. Suppl 1; p. A38
• Main Authors: Klempir, J, Keller, J, Vymazal, J, Syka, M, Stochl, J, Jech, R, Zidovska, J, Roth, J
• Format: Journal Article
• Language: English
• Published: 01-09-2014
• ISSN: 0022-3050
• DOI: 10.1136/jnnp-2014-309032.111

IntroductionIt has been hypothesised that changes of iron content in the brain may be involved in the pathogenesis of Huntington's disease (HD). The aim of our study was to detect whether the deposits of iron change during the disease progression.Subjects and methodsTo ascertain the hypothesis, we re-investigated 13 right-handed HD patients (mean interval 3.6 years, SD 0.5) using a 1.5 Tesla MR scanner and a multiple echo sequence with 16 echo-times was employed for T2 measurements. Following regions of interest were selected: head of caudate nucleus, putamen, globus pallidum (GP), frontal white matter (FWM) and frontal cortex.ResultsWe found a significant decrease of T2 relaxation time in the left GP (p < 0.01) and a significant increase of T2 relaxation time in the FWM of both lobes (p < 0.01) in HD patients compared to their first MR examination.The decrease of T2 relaxation time in the left GP inversely correlated with an expanded CAG repeat (p < 0.05). Moreover decrease of T2 relaxation time in the left (p < 0.01) GP inversely correlated with the interval of reinvestigation.DiscussionOur results suggest an additional significant increase in the iron (ferritin/hemosiderin) concentration in the GP of HD patients. Iron content is increasing with disease progression and genetic load. We suppose that the increase of T2 in the FWM may be related to the further disorganisation of white matter tracts due to the possible defect in axonal transport.AcknowledgementThis study was supported by a grant from Czech Ministry of Education, Research Program MSM0021620849, MSM0021620864, PRVOUK-P34, PRVOUK-P26/LF1/4 and by the Czech Ministry of Health: IGA MZ CR NT12282-5/2011 and by European Regional Development Fund - Project FNUSA-ICRC (No. CZ.1.05/1.1.00/02.0123).