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E08 Iron Content in Brain Increases with Progression of Huntington´s Disease: Longitudinal Study
  1. J Klempir1,
  2. J Keller2,3,
  3. J Vymazal2,
  4. M Syka2,4,
  5. J Štochl5,
  6. R Jech1,
  7. J Židovská6,
  8. J Roth1
  1. 1Department of Neurology and Center of Clinical Neuroscience, Charles University in Prague, 1st Faculty of Medicine and General University Hospital in Prague (Kateřinská 30, Prague, Czech Republic)
  2. 2Department of Radiodiagnostics, Na Homolce Hospital, Prague, Na Homolce Hospital Roentgenova 2, Czech Republic
  3. 3Department of Neurology, Third Faculty of Medicine, Charles University in Prague
  4. 4International Clinical Research Center, St. Anne´s University Hospital, Brno, Czech Republic
  5. 5Department of Psychiatry, University of Cambridge (Addenbrookes Hospital, Hills Road, Cambridge, CB29QQ)
  6. 6Institute of Biology and Medical Genetics, First Faculty of Medicine, Charles University in Prague and General University Hospital in Prague (Albertov 4, Prague, Czech Republic)


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).

  • Huntington’s disease
  • Brain iron
  • MRI
  • T2 relaxation
  • R2
  • Ferritin
  • Globus pallidum

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