{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,3]],"date-time":"2025-12-03T17:16:07Z","timestamp":1764782167817,"version":"build-2065373602"},"reference-count":24,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2018,10,17]],"date-time":"2018-10-17T00:00:00Z","timestamp":1539734400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100000627","name":"Guarantors of Brain","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000627","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000627","name":"Guarantors of Brain","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000627","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000288","name":"Royal Society","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100000288","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":[[2018,12]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Recent research has highlighted the role of the cerebellum in the pathophysiology of myoclonus\u2010dystonia syndrome as a result of mutations in the \u025b\u2010sarcoglycan gene (<jats:italic>DYT11<\/jats:italic>). Specifically, a cerebellar\u2010dependent saccadic adaptation task is dramatically impaired in this patient group.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objectives<\/jats:title><jats:p>The objective of this study was to investigate whether saccadic deficits coexist with impairments of limb adaptation to provide a potential mechanism linking cerebellar dysfunction to the movement disorder within symptomatic body regions.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Limb adaptation to visuomotor (visual feedback rotated by 30\u00b0) and forcefield (force applied by robot to deviate arm) perturbations were examined in 5 patients with <jats:italic>DYT11<\/jats:italic> and 10 aged\u2010matched controls.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Patients with <jats:italic>DYT11<\/jats:italic> successfully adapted to both types of perturbation. Modelled and averaged summary metrics that captured adaptation behaviors were equivalent to the control group across conditions.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>DYT11 is not characterized by a uniform deficit in adaptation. The previously observed large deficit in saccadic adaption is not reflected in an equivalent deficit in limb adaptation in symptomatic body regions. We suggest potential mechanisms at the root of this discordance and identify key research questions that need future study. \u00a9 2018 International Parkinson and Movement Disorder Society<\/jats:p><\/jats:sec>","DOI":"10.1002\/mds.27517","type":"journal-article","created":{"date-parts":[[2018,10,18]],"date-time":"2018-10-18T01:03:37Z","timestamp":1539824617000},"page":"1956-1961","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Delineating cerebellar mechanisms in DYT11 myoclonus\u2010dystonia"],"prefix":"10.1002","volume":"33","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-6132-2773","authenticated-orcid":false,"given":"Anna","family":"Sadnicka","sequence":"first","affiliation":[{"name":"Clinical and Movement Neurosciences UCL Queen Square Institute of Neurology  London UK"},{"name":"Motor Control and Movement Disorder Group, Institute of Molecular and Clinical Sciences St George's University of London  London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joseph M.","family":"Galea","sequence":"additional","affiliation":[{"name":"School of Psychology University of Birmingham  Birmingham UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jui\u2010Cheng","family":"Chen","sequence":"additional","affiliation":[{"name":"Clinical and Movement Neurosciences UCL Queen Square Institute of Neurology  London UK"},{"name":"Department of Neurology, China Medical University Hospital  Taiwan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Thomas T.","family":"Warner","sequence":"additional","affiliation":[{"name":"Clinical and Movement Neurosciences UCL Queen Square Institute of Neurology  London UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8185-286X","authenticated-orcid":false,"given":"Kailash P.","family":"Bhatia","sequence":"additional","affiliation":[{"name":"Clinical and Movement 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