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The structural correlates of functional deficits in early huntington's disease

Authors

  • Christine Delmaire,

    Corresponding author
    1. Department of Neuroradiology, CHRU Lille, France
    • Center for NeuroImaging Research—CENIR, Groupe Hospitalier Pitié-Salpêtrière, Paris, France
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  • Eve M. Dumas,

    1. Department of Neurology, Leiden University Medical Centre, 2300RC Leiden, The Netherlands
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  • Michael A. Sharman,

    1. Center for NeuroImaging Research—CENIR, Groupe Hospitalier Pitié-Salpêtrière, Paris, France
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  • Simon J.A. van den Bogaard,

    1. Department of Neurology, Leiden University Medical Centre, 2300RC Leiden, The Netherlands
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  • Romain Valabregue,

    1. Center for NeuroImaging Research—CENIR, Groupe Hospitalier Pitié-Salpêtrière, Paris, France
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  • Céline Jauffret,

    1. Department of Genetics and Cytogenetics, and INSERM UMR S679, APHP, Groupe Hospitalier Pitié-Salpêtrière, 75013 Paris, France
    2. Centre de Recherche de l'Institut du Cerveau et de la Moelle épinière, Université Pierre et Marie Curie—Paris 6, UMR-S975, Inserm U975, CNRS UMR 7225, Institut du Cerveau et de la Moelle épinière – ICM
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  • Damian Justo,

    1. Department of Genetics and Cytogenetics, and INSERM UMR S679, APHP, Groupe Hospitalier Pitié-Salpêtrière, 75013 Paris, France
    2. Centre de Recherche de l'Institut du Cerveau et de la Moelle épinière, Université Pierre et Marie Curie—Paris 6, UMR-S975, Inserm U975, CNRS UMR 7225, Institut du Cerveau et de la Moelle épinière – ICM
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  • Ralf Reilmann,

    1. Department of Neurology, University of Münster, 48129 Münster, Germany
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  • Julie C. Stout,

    1. School of Psychology, Psychiatry, and Psychological Medicine, Monash University, Clayton Campus, 3800, Victoria, Australia
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  • David Craufurd,

    1. University of Manchester, Manchester Academic Health Sciences Centre and Central Manchester University Hospitals NHS Foundation Trust, Manchester, M13 9WL, United Kingdom
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  • Sarah J. Tabrizi,

    1. UCL Institute of Neurology, University College London, London, WC1N 3BG, United Kingdom
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  • Raymund A.C. Roos,

    1. Department of Neurology, Leiden University Medical Centre, 2300RC Leiden, The Netherlands
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  • Alexandra Durr,

    1. Department of Genetics and Cytogenetics, and INSERM UMR S679, APHP, Groupe Hospitalier Pitié-Salpêtrière, 75013 Paris, France
    2. Centre de Recherche de l'Institut du Cerveau et de la Moelle épinière, Université Pierre et Marie Curie—Paris 6, UMR-S975, Inserm U975, CNRS UMR 7225, Institut du Cerveau et de la Moelle épinière – ICM
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  • Stéphane Lehéricy

    1. Center for NeuroImaging Research—CENIR, Groupe Hospitalier Pitié-Salpêtrière, Paris, France
    2. Centre de Recherche de l'Institut du Cerveau et de la Moelle épinière, Université Pierre et Marie Curie—Paris 6, UMR-S975, Inserm U975, CNRS UMR 7225, Institut du Cerveau et de la Moelle épinière – ICM
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Correspondence to: Christine Delmaire, M.D., Ph.D., Centre de NeuroImagerie de Recherche—CENIR, Institut du Cerveau et de la Moelle Epiniere—ICM, Hôpital de la Pitié-Salpêtrière, 47 boulevard de l'Hôpital, 75651 Paris CEDEX 13, France. E-mail: christine.delmaire@chru-lille.fr

Abstract

Neuropathological studies in Huntington disease (HD) have demonstrated neuronal loss in the striatum, as well as in other brain regions including the cortex. With diffusion tensor MRI we evaluated the hypothesis that the clinical dysfunction in HD is related to regionally specific lesions of circuit-specific cortico–basal ganglia networks rather than to the striatum only. We included 27 HD and 24 controls from the TRACK-HD Paris cohort. The following assessments were used: self-paced tapping tasks, trail B making test (TMT), University of Pennsylvania smell identification test (UPSIT), and apathy scores from the problem behaviors assessment. Group comparisons of fractional anisotropy and mean diffusivity and correlations were performed using voxel-based analysis. In the cortex, HD patients showed significant correlations between: (i) self paced tapping and mean diffusivity in the parietal lobe at 1.8 Hz and prefrontal areas at 3 Hz, (ii) UPSIT and mean diffusivity in the parietal, and median temporal lobes, the cingulum and the insula, and fractional anisotropy in the insula and the external capsule, (iii) TMT B and mean diffusivity in the white matter of the superior frontal, orbital, temporal, superior parietal and post central areas, and (iv) apathy and fractional anisotropy in the white matter of the rectus gyrus. In the basal ganglia, we found correlations between the self paced tapping, UPSIT, TMT tests, and mean diffusivity in the anterior part of the putamen and the caudate nucleus. In conclusion, disruption of motor, associative and limbic cortico-striatal circuits differentially contribute to the clinical signs of the disease. Hum Brain Mapp 34:2141–2153, 2013. © 2012 Wiley Periodicals, Inc.

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