Deep White Matter in Huntington's Disease |
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| Authors: | Owen Phillips Ferdinando Squitieri Cristina Sanchez-Castaneda Francesca Elifani Carlo Caltagirone Umberto Sabatini Margherita Di Paola |
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| Institution: | 1. Clinical and Behavioural Neurology Dept, IRCCS Santa Lucia Foundation, Rome, Italy.; 2. IRCSS Neuromed, Pozzilli, Italy.; 3. Radiology Dept, IRCCS Santa Lucia Foundation, Rome, Italy.; 4. Neuroscience Dept, University of Rome “Tor Vergata”, Rome, Italy.; Inserm U837, France, |
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| Abstract: | White matter (WM) abnormalities have already been shown in presymptomatic (Pre-HD) and symptomatic HD subjects using Magnetic Resonance Imaging (MRI). In the present study, we examined the microstructure of the long-range large deep WM tracts by applying two different MRI approaches: Diffusion Tensor Imaging (DTI) -based tractography, and T2*weighted (iron sensitive) imaging. We collected Pre-HD subjects (n = 25), HD patients (n = 25) and healthy control subjects (n = 50). Results revealed increased axial (AD) and radial diffusivity (RD) and iron levels in Pre-HD subjects compared to controls. Fractional anisotropy decreased between the Pre-HD and HD phase and AD/RD increased and although impairment was pervasive in HD, degeneration occurred in a pattern in Pre-HD. Furthermore, iron levels dropped for HD patients. As increased iron levels are associated with remyelination, the data suggests that Pre-HD subjects attempt to repair damaged deep WM years before symptoms occur but this process fails with disease progression. |
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