{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,29]],"date-time":"2026-09-29T09:33:23Z","timestamp":1790674403421,"version":"4.1.0"},"reference-count":31,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2018,9,15]],"date-time":"2018-09-15T00:00:00Z","timestamp":1536969600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/100005725","name":"CHDI Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100005725","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100004543","name":"China Scholarship Council","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100004543","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000266","name":"Engineering and Physical Sciences Research Council","doi-asserted-by":"publisher","award":["EP\/L022680\/1"],"award-info":[{"award-number":["EP\/L022680\/1"]}],"id":[{"id":"10.13039\/501100000266","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100010269","name":"Wellcome Trust","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100010269","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100010269","name":"Wellcome Trust","doi-asserted-by":"publisher","award":["200181\/Z\/15\/Z"],"award-info":[{"award-number":["200181\/Z\/15\/Z"]}],"id":[{"id":"10.13039\/100010269","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Annals of Neurology"],"published-print":{"date-parts":[[2018,10]]},"abstract":"<jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>Huntington's disease (HD) is a monogenic, fully penetrant neurodegenerative disorder, providing an ideal model for understanding brain changes occurring in the years prior to disease onset. Diffusion tensor imaging (DTI) studies show widespread white matter disorganization in the early premanifest stages (pre\u2010HD). However, although DTI has proved informative, it provides only limited information about underlying changes in tissue properties. Neurite orientation dispersion and density imaging (NODDI) is a novel magnetic resonance imaging (MRI) technique for characterizing axonal pathology more specifically, providing metrics that separately quantify axonal density and axonal organization. Here, we provide the first in vivo characterization of white matter pathology in pre\u2010HD using NODDI.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Diffusion\u2010weighted MRI data that support DTI and NODDI were acquired from 38 pre\u2010HD and 45 control participants. Using whole\u2010brain and region\u2010of\u2010interest analyses, NODDI metrics were compared between groups and correlated with clinical scores of disease progression. Whole\u2010brain changes in DTI metrics were also examined.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>The pre\u2010HD group displayed widespread reductions in axonal density compared with control participants; this correlated with measures of clinical disease progression in the body and genu of the corpus callosum. There was also evidence in the pre\u2010HD group of increased coherence of axonal packing in the white matter surrounding the basal ganglia.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Interpretation<\/jats:title>\n                    <jats:p>Our findings suggest that reduced axonal density is one of the major factors underlying white matter pathology in pre\u2010HD, coupled with altered local organization in areas surrounding the basal ganglia. NODDI metrics show promise in providing more specific information about the biological processes underlying HD and neurodegeneration per se. Ann Neurol 2018;84:497\u2013504<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/ana.25309","type":"journal-article","created":{"date-parts":[[2018,8,1]],"date-time":"2018-08-01T08:10:20Z","timestamp":1533111020000},"page":"497-504","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":82,"title":["In vivo characterization of white matter pathology in premanifest huntington's disease"],"prefix":"10.1002","volume":"84","author":[{"given":"Jiaying","family":"Zhang","sequence":"first","affiliation":[{"name":"Department of Computer Science and Centre for Medical Image Computing University College London  London United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah","family":"Gregory","sequence":"additional","affiliation":[{"name":"Huntington's Disease Research Centre, Institute of Neurology University College London  London United Kingdom"},{"name":"Wellcome Trust Centre for Neuroimaging, Institute of Neurology University College London  London United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rachael I.","family":"Scahill","sequence":"additional","affiliation":[{"name":"Huntington's Disease Research Centre, Institute of Neurology University College London  London United Kingdom"},{"name":"Department of Neurodegenerative Disease, Institute of Neurology University College London  London United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandra","family":"Durr","sequence":"additional","affiliation":[{"name":"ICM \u2013 Institut du Cerveau et de la Moelle Epini\u00e8re, INSERM U1127, CNRS UMR7225, Sorbonne Universit\u00e9s \u2013 UPMC Universit\u00e9 Paris VI UMR_S1127 and APHP, Genetic department Piti\u00e9\u2013Salp\u00eatri\u00e8re University Hospital  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David L.","family":"Thomas","sequence":"additional","affiliation":[{"name":"Neuroradiological Academic Unit, Department of Brain Repair and Rehabilitation, Institute of Neurology University College London  London United Kingdom"},{"name":"Leonard Wolfson Experimental Neurology Centre, Institute of Neurology University College London  London United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"St\u00e9phane","family":"Lehericy","sequence":"additional","affiliation":[{"name":"Neuroimaging Research Center, Brain and Spinal Cord Institute Pierre and Marie Curie University, Inserm UMR1127, CNRS 7225  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Geraint","family":"Rees","sequence":"additional","affiliation":[{"name":"Wellcome Trust Centre for Neuroimaging, Institute of Neurology University College London  London United Kingdom"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sarah 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