{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,16]],"date-time":"2026-07-16T15:11:35Z","timestamp":1784214695448,"version":"3.55.0"},"reference-count":42,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2024,1,24]],"date-time":"2024-01-24T00:00:00Z","timestamp":1706054400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["movementdisorders.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Movement Disord Clin Pract"],"published-print":{"date-parts":[[2024,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Cognitive changes in Huntington's disease (HD) precede motor manifestations. ENROLL\u2010HD platform includes four cognitive measures of information processing speed (IPS). Our group is eager to seek clinical markers in the life stage that is as close as possible to the age of onset (ie, the so called prodromal HD phase) because this is the best time for therapeutic interventions.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objectives<\/jats:title><jats:p>Our study aimed to test whether cognitive scores in prodromal ENROLL\u2010HD mutation carriers show the potential to predict the severity of motor and behavioral changes once HD became fully manifested.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>From the global ENROLL\u2010HD cohort of 21,343 participants, we first selected a premanifest Cohort#1 (ie, subjects with Total Motor Score (TMS) &lt;10 and Diagnostic Confidence Level (DCL) &lt;4, <jats:italic>N<\/jats:italic>\u2009=\u20091.222). From this cohort, we then focused on a prodromal Cohort#2 of subjects who were ascertained to phenoconvert into manifest HD at follow\u2010up visits (ie, subjects from 6\u2009\u2264\u2009TMS\u22649 and DCL\u2009&lt;4 to TMS\u226510 and DCL\u2009=\u20094, <jats:italic>n<\/jats:italic>\u2009=\u2009206).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The main results of our study showed that low IPS before phenoconversion in Cohort#2 predicted the severity of motor and behavioral manifestations. By combining the four IPS cognitive measures (eg, the Categorical Verbal Fluency Test; Stroop Color Naming Test; Stroop Word Reading; Symbol Digit Modalities Test), we generated a Composite Cognition Score (CCS). The lower the CCS score the higher the TMS and the apathy scores in the same longitudinally followed\u2010up patients after phenoconversion.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>CCS might represent a clinical instrument to predict the prognosis of mutation carriers who are close to manifesting HD.<\/jats:p><\/jats:sec>","DOI":"10.1002\/mdc3.13975","type":"journal-article","created":{"date-parts":[[2024,1,24]],"date-time":"2024-01-24T05:04:49Z","timestamp":1706072689000},"page":"363-372","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Prodromal Cognitive Changes as a Prognostic Indicator of Forthcoming Huntington's Disease Severity: A Retrospective Longitudinal Study"],"prefix":"10.1002","volume":"11","author":[{"given":"Simone","family":"Migliore","sequence":"first","affiliation":[{"name":"Huntington and Rare Diseases Unit, Fondazione IRCCS Casa Sollievo Della Sofferenza 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