{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,8]],"date-time":"2026-07-08T05:17:31Z","timestamp":1783487851122,"version":"3.55.0"},"reference-count":37,"publisher":"Wiley","issue":"7","license":[{"start":{"date-parts":[[2021,6,10]],"date-time":"2021-06-10T00:00:00Z","timestamp":1623283200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Brain and Behavior"],"published-print":{"date-parts":[[2021,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Introduction<\/jats:title>\n                    <jats:p>Behavioral and cognitive changes can be observed across all Huntington disease (HD) stages. Our multicenter and retrospective study investigated the association between cognitive and behavioral scale scores in manifest HD, at three different yearly timepoints.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We analyzed cognitive and behavioral domains by the Unified Huntington's Disease Rating Scale (UHDRS) and by the Problem Behaviors Assessment Short Form (PBA\u2010s), at three different yearly times of life (t0 or baseline, t1 after one year, t2 after two years), in 97 patients with manifest HD (mean age 48.62\u00a0\u00b1\u00a013.1), from three ENROLL\u2010HD Centers. In order to test the disease progression, we also examined patients\u2019 motor and functional changes by the UHDRS, overtime.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      The severity of apathy and of perseveration\/obsession was associated with the severity of the cognitive decline (\n                      <jats:italic>p<\/jats:italic>\n                      \u00a0&lt;\u00a0.0001), regardless of the yearly timepoint. The score of irritability significantly and positively correlated with perseveration errors in the verbal fluency test at t0 (\n                      <jats:italic>r<\/jats:italic>\n                      \u00a0=\u00a0.34;\n                      <jats:italic>p<\/jats:italic>\n                      \u00a0=\u00a0.001), while the psychosis significantly and negatively correlated with the information processing speed at t0 (\n                      <jats:italic>r<\/jats:italic>\n                      \u00a0=\u00a0\u2212.21;\n                      <jats:italic>p<\/jats:italic>\n                      \u00a0=\u00a0.038) and significantly and positively correlated with perseveration errors in the verbal fluency test at t1 (\n                      <jats:italic>r<\/jats:italic>\n                      \u00a0=\u00a0.35;\n                      <jats:italic>p<\/jats:italic>\n                      \u00a0&lt;\u00a0.0001).\n                    <\/jats:p>\n                    <jats:p>\n                      The disease progression was confirmed by the significant worsening of the UHDRS\u2010Total Motor Score (TMS) and of the UHDRS\u2010Total Functional Capacity (TFC) scale score after two\u2010year follow\u2010up (\n                      <jats:italic>p<\/jats:italic>\n                      \u00a0&lt;\u00a0.0001).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Although the progression of abnormal behavioral manifestations cannot be predicted in HD, the severity of apathy and perseveration\/obsessions are significantly associated with the severity of the cognitive function impairment, thus contributing, together, to the disease development and to patients\u2019 loss of independence, in addition to the neurological manifestations. This cognitive\u2010behavior pattern determines a common underlying deficit depending on a dysexecutive syndrome.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/brb3.2151","type":"journal-article","created":{"date-parts":[[2021,6,10]],"date-time":"2021-06-10T19:08:04Z","timestamp":1623352084000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":20,"title":["Cognitive and behavioral associated changes in manifest Huntington disease: A retrospective cross\u2010sectional study"],"prefix":"10.1002","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2665-231X","authenticated-orcid":false,"given":"Simone","family":"Migliore","sequence":"first","affiliation":[{"name":"Huntington and Rare Diseases Unit Fondazione IRCCS Casa Sollievo della Sofferenza Hospital San Giovanni Rotondo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giulia","family":"D\u2019Aurizio","sequence":"additional","affiliation":[{"name":"Department of Biotechnological and Applied Clinical Sciences University of L'Aquila L'Aquila Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sabrina","family":"Maffi","sequence":"additional","affiliation":[{"name":"Huntington and Rare Diseases Unit Fondazione IRCCS Casa Sollievo della Sofferenza Hospital San Giovanni Rotondo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Consuelo","family":"Ceccarelli","sequence":"additional","affiliation":[{"name":"Italian League for Research on Huntington and Related Diseases (LIRH) Foundation Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giovanni","family":"Ristori","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Mental Health and Sensory Organs Faculty of Medicine and Psychology Centre for Experimental Neurological Therapies S. Andrea Hospital Sapienza University Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7783-114X","authenticated-orcid":false,"given":"Silvia","family":"Romano","sequence":"additional","affiliation":[{"name":"Department of Neuroscience, Mental Health and Sensory Organs Faculty of Medicine and Psychology Centre for Experimental Neurological Therapies S. Andrea Hospital Sapienza University Rome Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anna","family":"Castaldo","sequence":"additional","affiliation":[{"name":"Department of Medical Genetics and Neurogenetics Fondazione IRCCS Istituto Neurologico Carlo Besta Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2405-3564","authenticated-orcid":false,"given":"Caterina","family":"Mariotti","sequence":"additional","affiliation":[{"name":"Department of Medical Genetics and Neurogenetics Fondazione IRCCS Istituto Neurologico Carlo Besta Milan Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giuseppe","family":"Curcio","sequence":"additional","affiliation":[{"name":"Department of Biotechnological and Applied Clinical Sciences University of L'Aquila L'Aquila Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-7397-1727","authenticated-orcid":false,"given":"Ferdinando","family":"Squitieri","sequence":"additional","affiliation":[{"name":"Huntington and Rare Diseases Unit Fondazione IRCCS Casa Sollievo della Sofferenza Hospital San Giovanni Rotondo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2021,6,10]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1159\/000112509"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1159\/000091523"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbr.2015.10.001"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1176\/appi.neuropsych.13070169"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1001\/archneur.1993.00540080076020"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.nicl.2019.101965"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.biopsych.2006.11.034"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1176\/jnp.2010.22.2.196"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2215-0366(16)30144-4"},{"key":"e_1_2_10_11_1","doi-asserted-by":"publisher","DOI":"10.1037\/neu0000190"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(18)30294-1"},{"key":"e_1_2_10_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.22117"},{"key":"e_1_2_10_14_1","doi-asserted-by":"publisher","DOI":"10.1155\/2013\/781029"},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.genhosppsych.2007.11.005"},{"key":"e_1_2_10_16_1","doi-asserted-by":"publisher","DOI":"10.1002\/mdc3.12388"},{"key":"e_1_2_10_17_1","volume-title":"Neuropsychological assessment","author":"Lezak M. 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