作者
NA Van de Zande, TH Massey, Duncan McLauchlan, A Pryce Roberts, R Zutt, Mark Wardle, GC Payne, Catherine Clenaghan, MAJ Tijssen, Anne Elizabeth Rosser, Kathryn J Peall
发表日期
2017/9
期刊
European journal of neurology
卷号
24
期号
9
页码范围
1140-1147
简介
Background and purpose
Huntington's disease (HD) is an autosomal dominant, neurodegenerative movement disorder, typically characterized by chorea. Dystonia is also recognized as part of the HD motor phenotype, although little work detailing its prevalence, distribution, severity and impact on functional capacity has been published to date.
Methods
Patients (>18 years of age) were recruited from the Cardiff (UK) HD clinic, each undergoing a standardized videotaped clinical examination and series of functional assessment questionnaires (Unified Huntington's Disease Rating Scale, Burke–Fahn–Marsden 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–Fahn–Marsden Dystonia Rating Scale and Unified Huntington's Disease Rating Scale. Statistical …
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