Issel Anne L. Lim1, 2, Xu Li1, 2, Jiri M.G. van Bergen3, Paul G. Unschuld4, Craig K. Jones1, 2, Russell L. Margolis4, 5, Christopher A. Ross4, 5, Peter C.M. van Zijl1, 2
1Radiology, Johns Hopkins University, Baltimore, MD, United States; 2F.M. Kirby Research Center for Functional Brain Imaging, Kennedy Krieger Institute, Baltimore, MD, United States; 3Biomedical Engineering, Eindhoven University of Technology, Eindhoven, Netherlands; 4Psychiatry and Behavioral Sciences, Johns Hopkins University, Baltimore, MD, United States; 5Neurology, Johns Hopkins University, Baltimore, MD, United States
Advanced stages of Huntington's Disease show increased brain iron concentration in the basal ganglia. However, neuropathology is not well characterized in prodromal subjects, who carry the huntingtin mutation but do not yet have sufficient signs or symptoms for clinical diagnosis. Qualitative Susceptibility Weighted Imaging (SWI) and Quantitative Susceptibility Mapping (QSM) can correlate magnetic susceptibility with iron concentration in gray matter (GM) structures. Using QSM atlas-based coregistration for automated efficient delineation of deep GM regions, we demonstrated increased paramagnetic signal in prodromal subjects compared to controls, possibly indicating an increase in iron concentration prior to the onset of overt Huntington's Disease.