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Dystonia is also recognized as part of the <jats:styled-content style=\"fixed-case\">HD<\/jats:styled-content> motor phenotype, although little work detailing its prevalence, distribution, severity and impact on functional capacity has been published to date.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Patients (&gt;18 years of age) were recruited from the Cardiff (UK) <jats:styled-content style=\"fixed-case\">HD<\/jats:styled-content> clinic, each undergoing a standardized videotaped clinical examination and series of functional assessment questionnaires (Unified Huntington's Disease Rating Scale, Burke\u2013Fahn\u2013Marsden Dystonia Rating Scale and modified version of the Toronto Western Spasmodic Torticollis Rating Scale). The presence and severity of dystonia were scored by four independent neurologists using the Burke\u2013Fahn\u2013Marsden Dystonia Rating Scale and Unified Huntington's Disease Rating Scale. Statistical analysis included Fisher's exact test, Wilcoxon test, <jats:sc>anova<\/jats:sc> and calculation of correlation coefficients where appropriate.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Forty\u2010eight patients [91% (48\/53)] demonstrated evidence of dystonia, with the highest prevalence in the left upper limb (<jats:italic>n<\/jats:italic> = 44, 83%), right upper limb most severely affected and eyes least affected. Statistically significant positive correlations (<jats:italic>P<\/jats:italic> &lt; 0.05) were observed between dystonia severity and increasing <jats:styled-content style=\"fixed-case\">HD<\/jats:styled-content> disease stage and motor disease duration. Deterioration in functional capacity also correlated with increasing dystonia severity. No significant relationship was observed with age at motor symptom onset or <jats:styled-content style=\"fixed-case\">CAG<\/jats:styled-content> repeat length.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>We report a high prevalence of dystonia in adult patients with <jats:styled-content style=\"fixed-case\">HD<\/jats:styled-content>, with worsening dystonia severity with increasing <jats:styled-content style=\"fixed-case\">HD<\/jats:styled-content> disease stage and motor disease duration. The recognition and management of dystonic symptoms in routine clinical practice will aid overall symptomatic treatment and functional improvement.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.13349","type":"journal-article","created":{"date-parts":[[2017,6,29]],"date-time":"2017-06-29T12:28:45Z","timestamp":1498739325000},"page":"1140-1147","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":22,"title":["Clinical characterization of dystonia in adult patients with Huntington's disease"],"prefix":"10.1111","volume":"24","author":[{"given":"N. A.","family":"van de Zande","sequence":"first","affiliation":[{"name":"Huntington's Disease Research Group Institute of Psychological Medicine and Clinical Neurosciences Cardiff UK"},{"name":"Faculty of Medical Science University of Groningen Groningen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"T. H.","family":"Massey","sequence":"additional","affiliation":[{"name":"Huntington's Disease Research Group Institute of Psychological Medicine and Clinical Neurosciences Cardiff UK"},{"name":"MRC Centre for Neuropsychiatric Genetics and Genomics Cardiff UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D.","family":"McLauchlan","sequence":"additional","affiliation":[{"name":"Huntington's Disease Research Group Institute of Psychological Medicine and Clinical Neurosciences Cardiff UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A.","family":"Pryce Roberts","sequence":"additional","affiliation":[{"name":"MRC Centre for Neuropsychiatric Genetics and Genomics Cardiff UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"R.","family":"Zutt","sequence":"additional","affiliation":[{"name":"Department of Neurology University Medical Centre of Groningen Groningen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Wardle","sequence":"additional","affiliation":[{"name":"Cardiff and Vale University Health Board University Hospital of Wales Cardiff"},{"name":"Wales Brain Repair and Intracranial Neurotherapeutics Unit (BRAIN) Cardiff University Cardiff"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"G. C.","family":"Payne","sequence":"additional","affiliation":[{"name":"Cardiff and Vale University Health Board University Hospital of Wales Cardiff"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"C.","family":"Clenaghan","sequence":"additional","affiliation":[{"name":"Huntington's Disease Research Group Institute of Psychological Medicine and Clinical Neurosciences Cardiff UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. A. J.","family":"Tijssen","sequence":"additional","affiliation":[{"name":"Department of Neurology University Medical Centre of Groningen Groningen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"A. 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