{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,6,26]],"date-time":"2026-06-26T21:31:12Z","timestamp":1782509472716,"version":"3.54.5"},"reference-count":34,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2017,10,1]],"date-time":"2017-10-01T00:00:00Z","timestamp":1506816000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"content-domain":{"domain":["elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Cortex"],"published-print":{"date-parts":[[2017,10]]},"DOI":"10.1016\/j.cortex.2017.07.017","type":"journal-article","created":{"date-parts":[[2017,8,3]],"date-time":"2017-08-03T14:30:15Z","timestamp":1501770615000},"page":"51-62","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":44,"special_numbering":"C","title":["Cognitive decline in Huntington's disease expansion gene carriers"],"prefix":"10.1016","volume":"95","author":[{"given":"Verena","family":"Baake","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert H.A.M.","family":"Reijntjes","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eve M.","family":"Dumas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer C.","family":"Thompson","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raymund A.C.","family":"Roos","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"issue":"4","key":"10.1016\/j.cortex.2017.07.017_bib1","doi-asserted-by":"crossref","first-page":"398","DOI":"10.1038\/ng0893-398","article-title":"The relationship between trinucleotide (CAG) repeat length and clinical features of Huntington's disease","volume":"4","author":"Andrew","year":"1993","journal-title":"Nature Genetics"},{"issue":"8","key":"10.1016\/j.cortex.2017.07.017_bib2","doi-asserted-by":"crossref","first-page":"1052","DOI":"10.1212\/WNL.56.8.1052","article-title":"Retest effects and cognitive decline in longitudinal follow-up of patients with early HD","volume":"56","author":"Bachoud-Levi","year":"2001","journal-title":"Neurology"},{"issue":"1","key":"10.1016\/j.cortex.2017.07.017_bib3","doi-asserted-by":"crossref","first-page":"209","DOI":"10.1016\/j.brainresrev.2008.04.001","article-title":"Magnetic resonance imaging as an approach towards identifying neuropathological biomarkers for Huntington's disease","volume":"58","author":"Bohanna","year":"2008","journal-title":"Brain Research Reviews"},{"issue":"1","key":"10.1016\/j.cortex.2017.07.017_bib4","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1176\/appi.neuropsych.13070169","article-title":"Reliability and factor structure of the short Problem Behaviors Assessment for Huntington's disease (PBA-s) in the TRACK-HD and REGISTRY studies","volume":"27","author":"Callaghan","year":"2015","journal-title":"The Journal of Neuropsychiatry and Clinical Neurosciences"},{"issue":"1","key":"10.1016\/j.cortex.2017.07.017_bib5","doi-asserted-by":"crossref","first-page":"1","DOI":"10.2741\/S355","article-title":"A review of cognition in Huntington's disease","volume":"5","author":"Dumas","year":"2013","journal-title":"Frontiers in bioscience (Scholar edition)"},{"key":"10.1016\/j.cortex.2017.07.017_bib6","doi-asserted-by":"crossref","first-page":"79","DOI":"10.1146\/annurev.clinpsy.032408.153550","article-title":"Advances in analysis of longitudinal data","volume":"6","author":"Gibbons","year":"2010","journal-title":"Annual Review of Clinical Psychology"},{"key":"10.1016\/j.cortex.2017.07.017_bib7","doi-asserted-by":"crossref","DOI":"10.1371\/currents.RRN1288","article-title":"Seven-year clinical follow-up of premanifest carriers of Huntington's disease","volume":"3","author":"Hart","year":"2011","journal-title":"PLoS Currents"},{"issue":"12","key":"10.1016\/j.cortex.2017.07.017_bib8","doi-asserted-by":"crossref","first-page":"1702","DOI":"10.1212\/01.WNL.0000098878.47789.BD","article-title":"Profile of cognitive progression in early Huntington's disease","volume":"61","author":"Ho","year":"2003","journal-title":"Neurology"},{"issue":"8","key":"10.1016\/j.cortex.2017.07.017_bib9","doi-asserted-by":"crossref","first-page":"1277","DOI":"10.1016\/S0028-3932(01)00217-2","article-title":"Verbal fluency in Huntington's disease: A longitudinal analysis of phonemic and semantic clustering and switching","volume":"40","author":"Ho","year":"2002","journal-title":"Neuropsychologia"},{"issue":"2","key":"10.1016\/j.cortex.2017.07.017_bib10","doi-asserted-by":"crossref","first-page":"136","DOI":"10.1002\/mds.870110204","article-title":"Unified Huntington's disease rating scale: Reliability and consistency","volume":"11","author":"Huntington Study Group","year":"1996","journal-title":"Movement Disorders: Official Journal of the Movement Disorder Society"},{"issue":"4","key":"10.1016\/j.cortex.2017.07.017_bib11","doi-asserted-by":"crossref","first-page":"267","DOI":"10.1111\/j.1399-0004.2004.00241.x","article-title":"A new model for prediction of the age of onset and penetrance for Huntington's disease based on CAG length","volume":"65","author":"Langbehn","year":"2004","journal-title":"Clinical Genetics"},{"issue":"2","key":"10.1016\/j.cortex.2017.07.017_bib12","doi-asserted-by":"crossref","first-page":"397","DOI":"10.1002\/ajmg.b.30992","article-title":"CAG-repeat length and the age of onset in Huntington disease (HD): A review and validation study of statistical approaches","volume":"153B","author":"Langbehn","year":"2010","journal-title":"American Journal of Medical Genetics. Part B, Neuropsychiatric Genetics: the Official Publication of the International Society of Psychiatric Genetics"},{"issue":"8","key":"10.1016\/j.cortex.2017.07.017_bib13","doi-asserted-by":"crossref","first-page":"935","DOI":"10.1007\/s00415-004-0461-9","article-title":"Cognitive changes in patients with Huntington's disease (HD) and asymptomatic carriers of the HD mutation\u2013a longitudinal follow-up study","volume":"251","author":"Lemiere","year":"2004","journal-title":"Journal of Neurology"},{"issue":"8","key":"10.1016\/j.cortex.2017.07.017_bib14","doi-asserted-by":"crossref","first-page":"901","DOI":"10.1080\/13803395.2011.574606","article-title":"Modeling longitudinal change in motor and cognitive processing speed in presymptomatic Huntington's disease","volume":"33","author":"Maroof","year":"2011","journal-title":"Journal of Clinical and Experimental Neuropsychology"},{"issue":"1","key":"10.1016\/j.cortex.2017.07.017_bib15","first-page":"21","article-title":"Brain imaging and cognitive dysfunctions in Huntington's disease","volume":"31","author":"Montoya","year":"2006","journal-title":"Journal of Psychiatry & Neuroscience: JPN"},{"issue":"12","key":"10.1016\/j.cortex.2017.07.017_bib16","doi-asserted-by":"crossref","first-page":"1409","DOI":"10.1136\/jnnp.2010.209668","article-title":"Observing Huntington's disease: The European Huntington's Disease Network's REGISTRY","volume":"82","author":"Orth","year":"2011","journal-title":"Journal of Neurology, Neurosurgery & Psychiatry"},{"issue":"11","key":"10.1016\/j.cortex.2017.07.017_bib17","doi-asserted-by":"crossref","first-page":"1233","DOI":"10.1136\/jnnp-2013-305114","article-title":"Cognitive decline in prodromal Huntington disease: Implications for clinical trials","volume":"84","author":"Paulsen","year":"2013","journal-title":"J Neurol Neurosurg Psychiatry"},{"issue":"5","key":"10.1016\/j.cortex.2017.07.017_bib18","doi-asserted-by":"crossref","first-page":"689","DOI":"10.1002\/ana.410410521","article-title":"CAG repeat number governs the development rate of pathology in Huntington's disease","volume":"41","author":"Penney","year":"1997","journal-title":"Annals of Neurology"},{"issue":"11","key":"10.1016\/j.cortex.2017.07.017_bib19","doi-asserted-by":"crossref","first-page":"1335","DOI":"10.1002\/mds.26011","article-title":"Diagnostic criteria for Huntington's disease based on natural history","volume":"29","author":"Reilmann","year":"2014","journal-title":"Movement Disorders: Official Journal of the Movement Disorder Society"},{"issue":"1","key":"10.1016\/j.cortex.2017.07.017_bib20","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1186\/1750-1172-5-40","article-title":"Huntington's disease: A clinical review","volume":"5","author":"Roos","year":"2010","journal-title":"Orphanet Journal of Rare Diseases"},{"issue":"1","key":"10.1016\/j.cortex.2017.07.017_bib21","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1212\/WNL.29.1.1","article-title":"Huntington disease: Clinical care and evaluation","volume":"29","author":"Shoulson","year":"1979","journal-title":"Neurology"},{"issue":"1","key":"10.1016\/j.cortex.2017.07.017_bib22","doi-asserted-by":"crossref","first-page":"33","DOI":"10.1017\/S1355617701711046","article-title":"Longitudinal evaluation of cognitive disorder in Huntington's disease","volume":"7","author":"Snowden","year":"2001","journal-title":"Journal of the International Neuropsychological Society: JINS"},{"issue":"2","key":"10.1016\/j.cortex.2017.07.017_bib23","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1076\/jcen.24.2.133.998","article-title":"Psychomotor, executive, and memory function in preclinical Huntington's disease","volume":"24","author":"Snowden","year":"2002","journal-title":"Journal of Clinical and Experimental Neuropsychology"},{"issue":"8","key":"10.1016\/j.cortex.2017.07.017_bib24","doi-asserted-by":"crossref","first-page":"1767","DOI":"10.1016\/j.neuropsychologia.2006.12.015","article-title":"Verbal episodic memory declines prior to diagnosis in Huntington's disease","volume":"45","author":"Solomon","year":"2007","journal-title":"Neuropsychologia"},{"issue":"13","key":"10.1016\/j.cortex.2017.07.017_bib25","doi-asserted-by":"crossref","first-page":"1830","DOI":"10.1002\/mds.22097","article-title":"Ten-year rate of longitudinal change in neurocognitive and motor function in prediagnosis Huntington disease","volume":"23","author":"Solomon","year":"2008","journal-title":"Movement Disorders: Official Journal of the Movement Disorder Society"},{"issue":"7","key":"10.1016\/j.cortex.2017.07.017_bib26","doi-asserted-by":"crossref","first-page":"687","DOI":"10.1136\/jnnp-2011-301940","article-title":"Evaluation of longitudinal 12 and 24 month cognitive outcomes in premanifest and early Huntington's disease","volume":"83","author":"Stout","year":"2012","journal-title":"Journal of Neurology, Neurosurgery & Psychiatry"},{"issue":"10","key":"10.1016\/j.cortex.2017.07.017_bib27","doi-asserted-by":"crossref","first-page":"1281","DOI":"10.1002\/mds.25964","article-title":"HD-CAB: A cognitive assessment battery for clinical trials in Huntington's disease 1,2,3","volume":"29","author":"Stout","year":"2014","journal-title":"Movement Disorders: Official Journal of the Movement Disorder Society"},{"issue":"9","key":"10.1016\/j.cortex.2017.07.017_bib28","doi-asserted-by":"crossref","first-page":"791","DOI":"10.1016\/S1474-4422(09)70170-X","article-title":"Biological and clinical manifestations of Huntington's disease in the longitudinal TRACK-HD study: Cross-sectional analysis of baseline data","volume":"8","author":"Tabrizi","year":"2009","journal-title":"Lancet Neurology"},{"issue":"7","key":"10.1016\/j.cortex.2017.07.017_bib29","doi-asserted-by":"crossref","first-page":"637","DOI":"10.1016\/S1474-4422(13)70088-7","article-title":"Predictors of phenotypic progression and disease onset in premanifest and early-stage Huntington's disease in the TRACK-HD study: Analysis of 36-month observational data","volume":"12","author":"Tabrizi","year":"2013","journal-title":"Lancet Neurology"},{"issue":"6","key":"10.1016\/j.cortex.2017.07.017_bib30","doi-asserted-by":"crossref","first-page":"971","DOI":"10.1016\/0092-8674(93)90585-E","article-title":"A novel gene containing a trinucleotide repeat that is expanded and unstable on Huntington's disease chromosomes","volume":"72","year":"1993","journal-title":"Cell"},{"issue":"1","key":"10.1016\/j.cortex.2017.07.017_bib31","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1016\/j.neuropsychologia.2009.09.002","article-title":"Automaticity and attention in Huntington's disease: When two hands are not better than one","volume":"48","author":"Thompson","year":"2010","journal-title":"Neuropsychologia"},{"issue":"6","key":"10.1016\/j.cortex.2017.07.017_bib32","doi-asserted-by":"crossref","first-page":"559","DOI":"10.1097\/00005072-198511000-00003","article-title":"Neuropathological classification of Huntington's disease","volume":"44","author":"Vonsattel","year":"1985","journal-title":"Journal of Neuropathology and Experimental Neurology"},{"key":"10.1016\/j.cortex.2017.07.017_bib33","series-title":"Huntington's disease conditional onset probability calculator","author":"Wild","year":"2013"},{"issue":"1","key":"10.1016\/j.cortex.2017.07.017_bib34","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1176\/jnp.15.1.7","article-title":"Cognitive and motor functioning in gene carriers for Huntington's disease: A baseline study","volume":"15","author":"Witjes-Ane","year":"2003","journal-title":"The Journal of Neuropsychiatry and Clinical Neurosciences"}],"container-title":["Cortex"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0010945217302423?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0010945217302423?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2018,8,29]],"date-time":"2018-08-29T19:53:57Z","timestamp":1535572437000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0010945217302423"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,10]]},"references-count":34,"alternative-id":["S0010945217302423"],"URL":"https:\/\/doi.org\/10.1016\/j.cortex.2017.07.017","relation":{},"ISSN":["0010-9452"],"issn-type":[{"value":"0010-9452","type":"print"}],"subject":[],"published":{"date-parts":[[2017,10]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Cognitive decline in Huntington's disease expansion gene carriers","name":"articletitle","label":"Article Title"},{"value":"Cortex","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.cortex.2017.07.017","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2017 Elsevier Ltd. All rights reserved.","name":"copyright","label":"Copyright"}]}}