RT Journal Article
SR Electronic
T1 E08 Iron Content in Brain Increases with Progression of Huntington´s Disease: Longitudinal Study
JF Journal of Neurology, Neurosurgery & Psychiatry
JO J Neurol Neurosurg Psychiatry
FD BMJ Publishing Group Ltd
SP A38
OP A38
DO 10.1136/jnnp-2014-309032.111
VO 85
IS Suppl 1
A1 Klempir, J
A1 Keller, J
A1 Vymazal, J
A1 Syka, M
A1 Štochl, J
A1 Jech, R
A1 Židovská, J
A1 Roth, J
YR 2014
UL http://jnnp.bmj.com/content/85/Suppl_1/A38.1.abstract
AB Introduction It 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 methods To 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. Results We 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. Discussion Our 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. Acknowledgement This 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 ČR NT12282–5/2011 and by European Regional Development Fund – Project FNUSA-ICRC (No. CZ.1.05/1.1.00/02.0123).