{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,10]],"date-time":"2026-10-10T14:04:38Z","timestamp":1791641078673,"version":"4.3.4"},"reference-count":46,"publisher":"BMJ","issue":"11","content-domain":{"domain":["bmj.com"],"crossmark-restriction":true},"short-container-title":["J Neurol Neurosurg Psychiatry"],"published-print":{"date-parts":[[2013,11]]},"DOI":"10.1136\/jnnp-2013-305114","type":"journal-article","created":{"date-parts":[[2013,8,3]],"date-time":"2013-08-03T05:06:28Z","timestamp":1375506388000},"page":"1233-1239","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":89,"title":["Cognitive decline in prodromal Huntington Disease: implications for clinical trials"],"prefix":"10.1136","volume":"84","author":[{"given":"Jane S","family":"Paulsen","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Megan M","family":"Smith","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeffrey D","family":"Long","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"the PREDICT HD investigators and coordinators of the Huntington Study Group","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2013,8,2]]},"reference":[{"key":"key-10.1136\/jnnp-2013-305114-1","doi-asserted-by":"crossref","first-page":"85","DOI":"10.2217\/fnl.09.78","article-title":"Early detection of Huntington's disease","volume":"5","author":"Paulsen","year":"2010","journal-title":"Future Neurol"},{"key":"key-10.1136\/jnnp-2013-305114-2","doi-asserted-by":"crossref","first-page":"883","DOI":"10.1001\/archneur.63.6.883","article-title":"Preparing for preventive clinical trials: the Predict-HD study","volume":"63","author":"Paulsen","year":"2006","journal-title":"Arch Neurol"},{"key":"key-10.1136\/jnnp-2013-305114-3","doi-asserted-by":"crossref","first-page":"59","DOI":"10.1001\/archneur.1995.00540250063014","article-title":"Longitudinal neuropsychological and genetic linkage analysis of persons at risk for Huntington's disease","volume":"52","author":"Giordani","year":"1995","journal-title":"Arch Neurol"},{"key":"key-10.1136\/jnnp-2013-305114-4","doi-asserted-by":"crossref","first-page":"621","DOI":"10.1136\/jnnp.61.6.621","article-title":"Neuropsychological stability over two years in asymptomatic carriers of the Huntington's disease mutation","volume":"61","author":"Campodonico","year":"1996","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"key-10.1136\/jnnp-2013-305114-5","doi-asserted-by":"crossref","first-page":"310","DOI":"10.1176\/appi.neuropsych.19.3.310","article-title":"Longitudinal evaluation of \u201cPresymptomatic\u201d carriers of Huntington's disease","volume":"19","author":"Witjes-Ane","year":"2007","journal-title":"J Neuropsychiatry Clin Neurosci"},{"key":"key-10.1136\/jnnp-2013-305114-6","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":"J Neurol Neurosurg Psychiatry"},{"key":"key-10.1136\/jnnp-2013-305114-7","doi-asserted-by":"crossref","first-page":"773","DOI":"10.1136\/jnnp.69.6.773","article-title":"Subtle changes among presymptomatic carriers of the Huntington's disease gene","volume":"69","author":"Kirkwood","year":"2000","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"key-10.1136\/jnnp-2013-305114-8","doi-asserted-by":"crossref","first-page":"658","DOI":"10.1212\/WNL.57.4.658","article-title":"Clinical markers of early disease in persons near onset of Huntington's disease","volume":"57","author":"Paulsen","year":"2001","journal-title":"Neurology"},{"key":"key-10.1136\/jnnp-2013-305114-9","doi-asserted-by":"crossref","first-page":"131","DOI":"10.1034\/j.1600-0404.2002.01192.x","article-title":"Longitudinal study evaluating neuropsychological changes in so-called asymptomatic carriers of the Huntington's disease mutation after 1 year","volume":"106","author":"Lemiere","year":"2002","journal-title":"Acta Neurol Scand"},{"key":"key-10.1136\/jnnp-2013-305114-10","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":"J Clin Exp Neuropsychol"},{"key":"key-10.1136\/jnnp-2013-305114-11","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":"J Neurol"},{"key":"key-10.1136\/jnnp-2013-305114-12","doi-asserted-by":"crossref","first-page":"466","DOI":"10.1176\/appi.neuropsych.20.4.466","article-title":"Neuropsychological deficits in Huntington's disease gene carriers and correlates of early \"conversion\"","volume":"20","author":"Brandt","year":"2008","journal-title":"J Neuropsychiatry Clin Neurosci"},{"key":"key-10.1136\/jnnp-2013-305114-13","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":"Mov Disord"},{"key":"key-10.1136\/jnnp-2013-305114-14","doi-asserted-by":"crossref","first-page":"379","DOI":"10.1136\/jnnp.2009.176982","article-title":"Progression in prediagnostic Huntington disease","volume":"81","author":"Rupp","year":"2010","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"key-10.1136\/jnnp-2013-305114-15","doi-asserted-by":"crossref","first-page":"RRN1288","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 Curr"},{"key":"key-10.1136\/jnnp-2013-305114-16","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":"J Clin Exp Neuropsychol"},{"key":"key-10.1136\/jnnp-2013-305114-17","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1016\/S1474-4422(10)70276-3","article-title":"Biological and clinical changes in premanifest and early stage Huntington's disease in the TRACK-HD study: the 12-month longitudinal analysis","volume":"10","author":"Tabrizi","year":"2011","journal-title":"Lancet Neurol"},{"key":"key-10.1136\/jnnp-2013-305114-18","doi-asserted-by":"crossref","first-page":"42","DOI":"10.1016\/S1474-4422(11)70263-0","article-title":"Potential endpoints for clinical trials in premanifest and early Huntington's disease in the TRACK-HD study: analysis of 24 month observational data","volume":"11","author":"Tabrizi","year":"2012","journal-title":"Lancet Neurol"},{"key":"key-10.1136\/jnnp-2013-305114-19","doi-asserted-by":"crossref","first-page":"136","DOI":"10.1212\/WNL.50.1.136","article-title":"A 24-week, double-blind, placebo-controlled trial of donepezil in patients with Alzheimer's disease. Donepezil Study Group","volume":"50","author":"Rogers","year":"1998","journal-title":"Neurology"},{"key":"key-10.1136\/jnnp-2013-305114-20","doi-asserted-by":"crossref","first-page":"1333","DOI":"10.1056\/NEJMoa013128","article-title":"Memantine in moderate-to-severe Alzheimer's disease","volume":"348","author":"Reisberg","year":"2003","journal-title":"N Engl J Med"},{"key":"key-10.1136\/jnnp-2013-305114-21","doi-asserted-by":"crossref","first-page":"633","DOI":"10.1001\/archpsyc.64.6.633","article-title":"Neurocognitive effects of antipsychotic medications in patients with chronic schizophrenia in the CATIE Trial","volume":"64","author":"Keefe","year":"2007","journal-title":"Arch Gen Psychiatry"},{"key":"key-10.1136\/jnnp-2013-305114-22","doi-asserted-by":"crossref","first-page":"1120","DOI":"10.1093\/schbul\/sbm083","article-title":"Recent advances in the development of novel pharmacological agents for the treatment of cognitive impairments in schizophrenia","volume":"33","author":"Buchanan","year":"2007","journal-title":"Schizophr Bull"},{"key":"key-10.1136\/jnnp-2013-305114-23","doi-asserted-by":"crossref","first-page":"218","DOI":"10.1016\/S0140-6736(07)60111-1","article-title":"Huntington's disease","volume":"369","author":"Walker","year":"2007","journal-title":"Lancet"},{"key":"key-10.1136\/jnnp-2013-305114-24","doi-asserted-by":"crossref","first-page":"136","DOI":"10.1002\/mds.870110204","article-title":"Huntington Study Group. Unified Huntington's Disease Rating Scale: reliability and consistency","volume":"11","year":"1996","journal-title":"Mov Disord"},{"key":"key-10.1136\/jnnp-2013-305114-25","doi-asserted-by":"crossref","first-page":"1763","DOI":"10.1002\/mds.22601","article-title":"Motor abnormalities in premanifest persons with Huntington's disease: the PREDICT-HD study","volume":"24","author":"Biglan","year":"2009","journal-title":"Mov Disord"},{"key":"key-10.1136\/jnnp-2013-305114-26","doi-asserted-by":"crossref","first-page":"12","DOI":"10.3389\/fnagi.2013.00012","article-title":"Refining the diagnosis of Huntington disease: the PREDICT-HD study","volume":"5","author":"Biglan","year":"2013","journal-title":"Front Aging Neurosci"},{"key":"key-10.1136\/jnnp-2013-305114-27","doi-asserted-by":"crossref","first-page":"751","DOI":"10.1002\/ajmg.b.31232","article-title":"Indexing disease progression at study entry with individuals at-risk for Huntington Disease","volume":"156","author":"Zhang","year":"2011","journal-title":"Am J Med Genet B Neuropsychiatr Genet"},{"key":"key-10.1136\/jnnp-2013-305114-28","volume-title":"Linear mixed models for longitudinal data","author":"Verbeke","year":"2000"},{"key":"key-10.1136\/jnnp-2013-305114-29","doi-asserted-by":"crossref","DOI":"10.1002\/9781119013563","volume-title":"Statistical analysis with missing data","author":"Little","year":"2002","edition":"2"},{"key":"key-10.1136\/jnnp-2013-305114-30","first-page":"267","article-title":"Information theory and an extension of the maximum likelihood principle","author":"Akaike","year":"1973"},{"key":"key-10.1136\/jnnp-2013-305114-31","doi-asserted-by":"crossref","first-page":"595","DOI":"10.1212\/01.wnl.0000335765.55346.fc","article-title":"Sample sizes for brain atrophy outcomes in trials for secondary progressive multiple sclerosis","volume":"72","author":"Altmann","year":"2009","journal-title":"Neurology"},{"key":"key-10.1136\/jnnp-2013-305114-32","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"},{"key":"key-10.1136\/jnnp-2013-305114-33","doi-asserted-by":"crossref","first-page":"629","DOI":"10.1162\/jocn.2003.15.5.629","article-title":"Task-set switching deficits in early-stage Huntington's disease: implications for basal ganglia function","volume":"15","author":"Aron","year":"2003","journal-title":"J Cogn Neurosci"},{"issue":"(Pt 5)","key":"key-10.1136\/jnnp-2013-305114-34","doi-asserted-by":"crossref","first-page":"1633","DOI":"10.1093\/brain\/119.5.1633","article-title":"Executive and mnemonic functions in early Huntington's disease","volume":"119","author":"Lawrence","year":"1996","journal-title":"Brain"},{"key":"key-10.1136\/jnnp-2013-305114-35","doi-asserted-by":"crossref","first-page":"567","DOI":"10.1080\/13803395.2010.541228","article-title":"The Trail Making Test in prodromal Huntington disease: contributions of disease progression to test performance","volume":"33","author":"O'Rourke","year":"2011","journal-title":"J Clin Exp Neuropsychol"},{"key":"key-10.1136\/jnnp-2013-305114-36","first-page":"1715","article-title":"fMRI biomarker of early neuronal dysfunction in presymptomatic Huntington's Disease","volume":"25","author":"Paulsen","year":"2004","journal-title":"AJNR Am J Neuroradiol"},{"key":"key-10.1136\/jnnp-2013-305114-37","doi-asserted-by":"crossref","first-page":"758","DOI":"10.1017\/S1355617707071214","article-title":"fMRI detection of early neural dysfunction in preclinical Huntington's disease","volume":"13","author":"Zimbelman","year":"2007","journal-title":"J Int Neuropsychol Soc"},{"key":"key-10.1136\/jnnp-2013-305114-38","doi-asserted-by":"crossref","first-page":"261","DOI":"10.1007\/s11065-010-9143-9","article-title":"The cerebellum and basal ganglia are interconnected","volume":"20","author":"Bostan","year":"2010","journal-title":"Neuropsychol Rev"},{"key":"key-10.1136\/jnnp-2013-305114-39","doi-asserted-by":"crossref","first-page":"1274","DOI":"10.1093\/cercor\/bhp194","article-title":"Neural modulation of temporal encoding, maintenance, and decision processes","volume":"20","author":"Harrington","year":"2010","journal-title":"Cereb Cortex"},{"key":"key-10.1136\/jnnp-2013-305114-40","doi-asserted-by":"crossref","first-page":"1177","DOI":"10.1016\/j.neuroimage.2006.01.041","article-title":"From preparation to online control: reappraisal of neural circuitry mediating internally generated and externally guided actions","volume":"31","author":"Elsinger","year":"2006","journal-title":"Neuroimage"},{"key":"key-10.1136\/jnnp-2013-305114-41","doi-asserted-by":"crossref","first-page":"612","DOI":"10.1136\/jnnp-2011-301732","article-title":"Cognitive domains that predict time to diagnosis in prodromal Huntington disease","volume":"83","author":"Harrington","year":"2012","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"key-10.1136\/jnnp-2013-305114-42","doi-asserted-by":"crossref","first-page":"5528","DOI":"10.1523\/JNEUROSCI.17-14-05528.1997","article-title":"Distributed neural systems underlying the timing of movements","volume":"17","author":"Rao","year":"1997","journal-title":"J Neurosci"},{"key":"key-10.1136\/jnnp-2013-305114-43","doi-asserted-by":"crossref","first-page":"357","DOI":"10.1146\/annurev.ne.09.030186.002041","article-title":"Parallel organization of functionally segregated circuits linking basal ganglia and cortex","volume":"9","author":"Alexander","year":"1986","journal-title":"Annu Rev Neurosci"},{"key":"key-10.1136\/jnnp-2013-305114-44","doi-asserted-by":"crossref","first-page":"8452","DOI":"10.1073\/pnas.1000496107","article-title":"The basal ganglia communicate with the cerebellum","volume":"107","author":"Bostan","year":"2010","journal-title":"Proc Natl Acad Sci USA"},{"key":"key-10.1136\/jnnp-2013-305114-45","doi-asserted-by":"crossref","first-page":"474","DOI":"10.1007\/s11910-011-0215-x","article-title":"Cognitive impairment in Huntington disease: diagnosis and treatment","volume":"11","author":"Paulsen","year":"2011","journal-title":"Curr Neurol Neurosci Rep"},{"key":"key-10.1136\/jnnp-2013-305114-46","doi-asserted-by":"crossref","first-page":"250","DOI":"10.1038\/nrneurol.2012.67","article-title":"Neurodegenerative disease: establishing a clinical trial battery for Huntington disease","volume":"8","author":"Paulsen","year":"2012","journal-title":"Nat Rev Neurol"}],"container-title":["Journal of Neurology, Neurosurgery & Psychiatry"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.1136\/jnnp-2013-305114","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,9,25]],"date-time":"2020-09-25T17:52:17Z","timestamp":1601056337000},"score":1,"resource":{"primary":{"URL":"https:\/\/jnnp.bmj.com\/lookup\/doi\/10.1136\/jnnp-2013-305114"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,8,2]]},"references-count":46,"journal-issue":{"issue":"11","published-online":{"date-parts":[[2013,10,9]]},"published-print":{"date-parts":[[2013,11]]}},"alternative-id":["10.1136\/jnnp-2013-305114"],"URL":"https:\/\/doi.org\/10.1136\/jnnp-2013-305114","relation":{},"ISSN":["0022-3050","1468-330X"],"issn-type":[{"value":"0022-3050","type":"print"},{"value":"1468-330X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,8,2]]}}}