title: Deep white matter in Huntington's disease.
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.
authors.affiliation: Clinical and Behavioural Neurology Dept, IRCCS Santa Lucia Foundation, Rome, Italy.
authors.forename: Owen
authors.initials: O
authors.lastname: Phillips
authors.affiliation: IRCSS Neuromed, Pozzilli, Italy.
authors.forename: Ferdinando
authors.initials: F
authors.lastname: Squitieri
authors.affiliation: Radiology Dept, IRCCS Santa Lucia Foundation, Rome, Italy.
authors.forename: Cristina
authors.initials: C
authors.lastname: Sanchez-Castaneda
authors.affiliation: IRCSS Neuromed, Pozzilli, Italy.
authors.forename: Francesca
authors.initials: F
authors.lastname: Elifani
authors.affiliation: Clinical and Behavioural Neurology Dept, IRCCS Santa Lucia Foundation, Rome, Italy; Neuroscience Dept, University of Rome "Tor Vergata", Rome, Italy.
authors.forename: Carlo
authors.initials: C
authors.lastname: Caltagirone
authors.affiliation: Radiology Dept, IRCCS Santa Lucia Foundation, Rome, Italy.
authors.forename: Umberto
authors.initials: U
authors.lastname: Sabatini
authors.affiliation: Clinical and Behavioural Neurology Dept, IRCCS Santa Lucia Foundation, Rome, Italy.
authors.forename: Margherita
authors.initials: M
authors.lastname: Di Paola
mesh_terms: D000328:Adult; D016022:Case-Control Studies; D054022:Cerebrum / Q000473:pathology; D003710:Demography; D056324:Diffusion Tensor Imaging; D005260:Female; D006801:Humans; D006816:Huntington Disease / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D066127:White Matter / Q000473:pathology*