{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T21:25:15Z","timestamp":1790889915715,"version":"4.1.0"},"reference-count":39,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2014,8,27]],"date-time":"2014-08-27T00:00:00Z","timestamp":1409097600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Movement Disorders"],"published-print":{"date-parts":[[2014,9,15]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Huntington's disease (HD) is currently diagnosed based on the presence of motor signs indicating 99% \u201cdiagnostic confidence\u201d for HD. Recent advances in the understanding of HD natural history and neurobiology indicate that disease\u2010related brain changes begin at least 12 to 15 years before the formal diagnosis based on motor onset. Furthermore, subtle motor dysfunction, cognitive changes, and behavioral alterations are often seen before diagnosis made according to the current criteria. As disease\u2010modifying treatments are developed, likely beginning therapy early will be desirable. We therefore suggest that expanded diagnostic criteria for HD should be adapted to better reflect the natural history of the disease, to enable the conduct of clinical trials in premanifest subjects targeting prevention of neurodegeneration, and to facilitate earlier symptomatic treatment. We propose a new set of criteria for HD diagnostic categories in the International Classification of Diseases that reflect our current understanding of HD natural history and pathogenesis. Based on defined criteria, for example, the Diagnostic Confidence Level and the Total Functional Capacity scales of the Unified Huntington's Disease Rating Scale, HD should be divided in the categories \u201cgenetically confirmed\u201d with the subcategories \u201cpresymptomatic,\u201d \u201cprodromal,\u201d and \u201cmanifest\u201d and \u201cnot genetically confirmed\u201d subdivided into \u201cclinically at risk,\u201d \u201cclinically prodromal,\u201d and \u201cclinically manifest.\u201d \u00a9 2014 International Parkinson and Movement Disorder Society<\/jats:p>","DOI":"10.1002\/mds.26011","type":"journal-article","created":{"date-parts":[[2014,8,27]],"date-time":"2014-08-27T10:25:33Z","timestamp":1409135133000},"page":"1335-1341","source":"Crossref","is-referenced-by-count":201,"title":["Diagnostic criteria for Huntington's disease based on natural 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