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Front Neurosci. 2018 Nov 5;12:805. doi: 10.3389/fnins.2018.00805. eCollection 2018.

Altered Intracortical T1-Weighted/T2-Weighted Ratio Signal in Huntington's Disease.

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McMaster Integrative Neuroscience Discovery and Study Program, McMaster University, Hamilton, ON, Canada.
Huntington's Disease Centre, University College London Institute of Neurology, National Hospital for Neurology and Neurosurgery, London, United Kingdom.
Department of Medical Genetics, The University of British Columbia, Vancouver, BC, Canada.
Department of Neurology, Leiden University Medical Center, Leiden, Netherlands.
INSERM U1127, CNRS UMR7225, UMR_S1127, UPMC Université Paris VI, Institut du Cerveau et de la Moelle Epinière, Sorbonne University, Paris, France.
APHP, Department of Genetics, Pitié-Salpêtrière University Hospital, Paris, France.
Department of Psychology, Neuroscience and Behaviour, McMaster University, Hamilton, ON, Canada.


Huntington's disease (HD) is a genetic neurodegenerative disorder that is characterized by neuronal cell death. Although medium spiny neurons in the striatum are predominantly affected, other brain regions including the cerebral cortex also degenerate. Previous structural imaging studies have reported decreases in cortical thickness in HD. Here we aimed to further investigate changes in cortical tissue composition in vivo in HD using standard clinical T1-weighted (T1W) and T2-weighted (T2W) magnetic resonance images (MRIs). 326 subjects from the TRACK-HD dataset representing healthy controls and four stages of HD progression were analyzed. The intracortical T1W/T2W intensity was sampled in the middle depth of the cortex over 82 regions across the cortex. While these previously collected images were not optimized for intracortical analysis, we found a significant increase in T1W/T2W intensity (p < 0.05 Bonferroni-Holm corrected) beginning with HD diagnosis. Increases in ratio intensity were found in the insula, which then spread to ventrolateral frontal cortex, superior temporal gyrus, medial temporal gyral pole, and cuneus with progression into the most advanced HD group studied. Mirroring past histological reports, this increase in the ratio image intensity may reflect disease-related increases in myelin and/or iron in the cortex. These findings suggest that future imaging studies are warranted with imaging optimized to more sensitively and specifically assess which features of cortical tissue composition are abnormal in HD to better characterize disease progression.


Huntington’s disease; MRI; cerebral cortex; myelin; neurodegeneration

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