{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T15:01:24Z","timestamp":1790348484881,"version":"4.1.0"},"reference-count":37,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2026,2,11]],"date-time":"2026-02-11T00:00:00Z","timestamp":1770768000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"},{"start":{"date-parts":[[2026,2,11]],"date-time":"2026-02-11T00:00:00Z","timestamp":1770768000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"funder":[{"DOI":"10.13039\/501100000780","name":"European Commission","doi-asserted-by":"publisher","award":["101156595"],"award-info":[{"award-number":["101156595"]}],"id":[{"id":"10.13039\/501100000780","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["movementdisorders.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Movement Disorders"],"published-print":{"date-parts":[[2026,4]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>Neuropsychological deficits have been observed in patients with cerebellar damage, but never thoroughly investigated in autosomal recessive spastic ataxia of Charlevoix\u2010Saguenay (ARSACS).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>The goal is the characterization of presence, severity, and profile of neuropsychological deficits in ARSACS using the cerebellar cognitive\u2010affective syndrome (CCAS) scale.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>Prospective study including a discovery cohort from Saguenay\/Canada (n\u2009=\u200931, median [inter\u2010quartile range] age: 57 [54\u201362] years), and a validation cohort from T\u00fcbingen, Germany (n\u2009=\u200917, 35 [21\u201343] years) with matched controls (n\u2009=\u200919).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      All ARSACS patients failed in multiple CCAS\u2010related subtests and exceeded cutoffs for \u201cdefinite CCAS.\u201d Even the younger validation cohort failed more subtests than controls (5 [3\u20137] vs. 1 [1\u20132],\n                      <jats:italic>P<\/jats:italic>\n                      \u2009&lt;\u20090.001) and had lower CCAS total scores (81 [67\u201386] vs. 101 [91\u2013106],\n                      <jats:italic>P<\/jats:italic>\n                      \u2009&lt;\u20090.001). Total scores worsened in the older discovery cohort (40 [25\u201352],\n                      <jats:italic>P<\/jats:italic>\n                      \u2009&lt;\u20090.001) and correlated with age\/disease duration (\u03c1\u2009=\u2009\u22120.575,\n                      <jats:italic>P<\/jats:italic>\n                      \u2009&lt;\u20090.001) and ataxia severity (Scale for the Assessment and Rating of Ataxia: \u03c1\u2009=\u2009\u22120.527,\n                      <jats:italic>P<\/jats:italic>\n                      \u2009=\u20090.003).\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusions<\/jats:title>\n                    <jats:p>\n                      Neuropsychological deficits consistent with CCAS are consistent in ARSACS, present early, and progress in the disease course. \u00a9 2026 The Author(s).\n                      <jats:italic>Movement Disorders<\/jats:italic>\n                      published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/mds.70201","type":"journal-article","created":{"date-parts":[[2026,2,11]],"date-time":"2026-02-11T10:55:36Z","timestamp":1770807336000},"page":"1034-1040","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["The Cerebellar Cognitive\u2010Affective Syndrome Scale Reveals Consistent, Early, and Progressive Neuropsychological Deficits in Autosomal\u2010Recessive Spastic Ataxia of Charlevoix\u2010Saguenay: A Large International Cross\u2010Sectional Study"],"prefix":"10.1002","volume":"41","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1957-8518","authenticated-orcid":false,"given":"Julie","family":"Fortin","sequence":"first","affiliation":[{"name":"Groupe de Recherche Interdisciplinaire sur les Maladies Neuromusculaires (GRIMN), Centre 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