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Imaging
I05 Changes in iron concentrations in Huntington's disease
  1. E Dumas1,
  2. M Versluis2,
  3. S J A van den Bogaard1,
  4. A Webb2,
  5. J van der Grond3,
  6. R A C Roos1
  1. 1Department of Neurology, Leiden University Medical Centre, Leiden, The Netherlands
  2. 2CJ Gorter Centre for High Field MRI, Leiden University Medical Centre, Leiden, The Netherlands
  3. 3Department of Radiology, Leiden University Medical Centre, Leiden, The Netherlands

Abstract

Background It has been suggested that increased iron levels in subcortical structures is one of the causal factors of the symptoms of Huntington's disease (HD). Magnetic field correlation (MFC) is an imaging technique sensitive to magnetic field inhomogeneities as a result of iron accumulations. MFC is not sensitive to changes in water concentration due, for example, to demyelination and can therefore be used for investigating and quantifying the iron depositions in HD. Iron imaging is novel in HD and should give insight into disease processes.

Aim To examine regional iron accumulation in HD and the relation to clinical measures.

Methods 28 HD patients (mean age 49 years), 21 premanifest gene carriers (mean age 45 years) and 25 healthy controls (mean age 50 years), from the Leiden site of the TRACK-HD study, underwent 3T MRI including high resolution 3D T1 weighted and asymmetric spin echo (ASE) sequences. From these ASE scans MFC maps were calculated. Post-processing involved automated registration to T1 scans and deep grey matter structure segmentation with FIRST. MFC values were compared between groups and related to clinical measures.

Results The MFC in the caudate nucleus (p<0.05) and putamen (p<0.005) of HD patients was significantly higher than in control subjects. The MFC in the nucleus lentiformus (pallidum+putamen) was also higher in HD compared with controls (p<0.005). The pallidum itself showed a trend (p=0.07) towards higher values in HD. No significant differences were found between premanifest gene carriers and controls. In all subjects, MFC values demonstrated a significant correlation (corrected for age and education) between these structures and such measures as the UHDRS, SDMT, TMT-B, an emotion recognition and a smell identification test.

Conclusion Patients with HD have higher magnetic field inhomogeneities in deep grey matter structures which are likely to be caused by increases in iron concentration. These changes observed with ASE measurements have a significant association with clinical measures.

  • iron depositions
  • magnetic field inhomogeneities
  • imaging

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