Journal Article10.1002/MDS.870110204
Unified huntington’s disease rating scale: Reliability and consistency
Karl Kieburtz,John B. Penney,Peter Corno,Neal G. Ranen,Ira Shoulson,Andrew Feigin,Davi Abwender,J. Timothy Greenarnyre,Donald S. Higgins,Frederick J. Marshall,Joshua L. Goldstein,Kimberly Steinberg,Charles Shih,Irene H. Richard,Charlyne Hickey,Carol Zimmerman,Constance Orme,Kathy Claude,David Oakes,Daniel S. Sax,Anthony Kim,Steven M. Hersch,Randi Jones,Alexander P. Auchus,David B. Olsen,Cheryl Bissey-Black,Allen Rubin,Rose Schwartz,Richard Dubinsky,William Mallonee,Carolyn Gray,Nan Godfrey,Greg Suter,Kathleen M. Shannon,Glenn T. Stebbins,Jean A. Jaglin,Karen Marder,Stuart Taylor,Elan D. Louis,Carol Moskowitz,Deborah Zeck Thorne,Naomi Zubin,Nancy S. Wexler,Michael R. Swenson,Jane S. Paulsen,Neal R. Swerdlow,Roger L. Albin,Christine Wernette,Francis O. Walker,Vicki Hunt +49 more
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TL;DR: The limited longitudinal database indicates that the UHDRS may be useful for tracking changes in the clinical features of HD over time and there was an excellent degree of interrater reliability for the motor scores.
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Abstract: The Unified Huntington's disease Rating Scale (UHDRS) was developed as a clinical rating scale to assess four domains of clinical performance and capacity in HD: motor function, cognitive function, behavioral abnormalities, and functional capacity. We assessed the internal consistency and the intercorrelations for the four domains and examined changes in ratings over time. We also performed an interrater reliability study of the motor assessment. We found there was a high degree of internal consistency within each of the domains of the UHDRS and that there were significant intercorrelations between the domains of the UHDRS, with the exception of the total behavioral score. There was an excellent degree of interrater reliability for the motor scores. Our limited longitudinal database indicates that the UHDRS may be useful for tracking changes in the clinical features of HD over time. The UHDRS assesses relevant clinical features of HD and appears to be appropriate for repeated administration during clinical studies.
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Citations
Multiple Wearable Sensors in Parkinson and Huntington Disease Individuals: A Pilot Study in Clinic and at Home
Jamie L. Adams,Karthik Dinesh,Mulin Xiong,Christopher G. Tarolli,Saloni Sharma,Nirav Sheth,Alexander J. Aranyosi,William Zhu,Steven Goldenthal,Kevin M. Biglan,E. Ray Dorsey,Gaurav Sharma +11 more
- 17 Aug 2017
TL;DR: Among individuals with movement disorders, the use of wearable sensors in clinic and at home was feasible and well-received, and these sensors can identify statistically significant differences in activity profiles between individuals with movements disorders and those without.
Huntington's disease.
TL;DR: Effective intervention by clinicians is possible in terms of providing patients and families with accurate information about the disease, counseling them about availability of genetic testing at specialized centers, and in giving them sound advice regarding work, driving, relationships, finances, research participation, and support groups.
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Scale for the assessment and rating of ataxia: development of a new clinical scale.
T. Schmitz-Hübsch,S. Tezenas du Montcel,Laszlo Baliko,José Berciano,S Boesch,Chantal Depondt,Paola Giunti,C. Globas,Jon Infante,J-S Kang,Berry Kremer,Christian Mariotti,Béla Melegh,Massimo Pandolfo,Maryla Rakowicz,P Ribai,Rafał Rola,Ludger Schöls,Sandra Szymanski,B.P.C. van de Warrenburg,Alexandra Durr,Thomas Klockgether,Roberto Fancellu +22 more
TL;DR: The Scale for the Assessment and Rating of Ataxia is a reliable and valid measure of ataxia, making it an appropriate primary outcome measure for clinical trials.
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Huntington's disease: from molecular pathogenesis to clinical treatment
TL;DR: Predictive genetic testing and findings of neuroimaging studies show that Huntington's disease is emerging as a model for strategies to develop therapeutic interventions, not only to slow progression of manifest disease but also to delay, or ideally prevent, its onset.
Huntington's disease: a clinical review
TL;DR: Huntington disease is a rare neurodegenerative disorder of the central nervous system characterized by unwanted choreatic movements, behavioral and psychiatric disturbances and dementia, which results in patients requiring full-time care, and finally death.
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TL;DR: In the last twenty years, the accurate evaluation of extrapyramidal disorders has become essential to assess the efficacy of an increasing number of potentially useful drugs as discussed by the authors, and since the introduction of levodopa, Parkinson's disease has attracted the most attention for developing assessment techniques.
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The measurement of abnormal movement: Methods developed for Huntington's Disease.
TL;DR: The mechanized tests may be useful for screening for motor disorders in the population: they are non-specific, portable and do not require the services of an experienced clinician.
Factors Associated With Slow Progression in Huntington's Disease
Richard H. Myers,Daniel S. Sax,Walter J. Koroshetz,Carol A. Mastromauro,L. A. Cupples,Dan K. Kiely,F K Pettengill,Edward D. Bird +7 more
TL;DR: Men tended to have a slower disease progression than did women, and this was particularly evident among men inheriting Huntington's disease from affected mothers, and neither the butyrophenone haloperidol nor the tricyclic antidepressant imipramine were related to rate of progression.
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