Noelle E Carlozzi1, Stephen G Schilling1,2, Nicholas R Boileau1, Kelvin L Chou3, Joel S Perlmutter4, Samuel Frank5, Michael K McCormack6,7, Julie C Stout8, Jane S Paulsen9, Jin-Shei Lai10, Praveen Dayalu3. 1. Department of Physical Medicine and Rehabilitation, University of Michigan, Ann Arbor, Michigan, USA. 2. Institute for Social Research, University of Michigan, Ann Arbor, Michigan, USA. 3. Department of Neurology, University of Michigan, Ann Arbor, Michigan, USA. 4. Department of Neurology, Radiology, Neuroscience, Physical Therapy and Occupational Therapy, Washington University in St. Louis, St. Louis, Missouri, USA. 5. Department of Neurology, Beth Israel Deaconess Medical Center, Boston, Massachusetts, USA. 6. Department of Psychiatry, Rutgers-Robert Wood Johnson Medical School, Piscataway, New Jersey, USA. 7. Department of Pathology, Rowan-SOM (School of Medicine), Stratford, New Jersey, USA. 8. School of Psychological Sciences, Monash University, Clayton, Victoria, Australia. 9. Department of Psychiatry, Neurology, and Psychological and Brain Sciences, The University of Iowa, Iowa City, Iowa, USA. 10. Department of Medical Social Sciences, Northwestern University, Chicago, Illinois, USA.
Abstract
PURPOSE: This study examined the relationships between different aspects of motor dysfunction (chorea, dystonia, rigidity, incoordination, oculomotor dysfunction, dysarthria, and gait difficulties) and functional status in persons with Huntington's disease. METHODS: A total of 527 persons with Huntington's disease completed the Unified Huntington's Disease Rating Scale motor, total functional capacity, and functional assessments. RESULTS: Confirmatory factor analysis indicated that a 4-factor model provided a better model fit than the existing 5-factor model. Exploratory factor analysis identified the following 4 factors from the motor scale: dystonia, chorea, rigidity, and a general motor factor. Regression indicated that dystonia (β = -0.47 and -0.79) and rigidity (β = -0.28 and -0.59) had strong associations with function, whereas chorea had modest correlations (β = -0.16 and -0.15). CONCLUSIONS: Dystonia and rigidity have stronger relationships with functional status than chorea in persons with Huntington's disease. The findings underscore the need for further research regarding the effects of dystonia and rigidity on functioning.
PURPOSE: This study examined the relationships between different aspects of motor dysfunction (chorea, dystonia, rigidity, incoordination, oculomotor dysfunction, dysarthria, and gait difficulties) and functional status in persons with Huntington's disease. METHODS: A total of 527 persons with Huntington's disease completed the Unified Huntington's Disease Rating Scale motor, total functional capacity, and functional assessments. RESULTS: Confirmatory factor analysis indicated that a 4-factor model provided a better model fit than the existing 5-factor model. Exploratory factor analysis identified the following 4 factors from the motor scale: dystonia, chorea, rigidity, and a general motor factor. Regression indicated that dystonia (β = -0.47 and -0.79) and rigidity (β = -0.28 and -0.59) had strong associations with function, whereas chorea had modest correlations (β = -0.16 and -0.15). CONCLUSIONS: Dystonia and rigidity have stronger relationships with functional status than chorea in persons with Huntington's disease. The findings underscore the need for further research regarding the effects of dystonia and rigidity on functioning.
Authors: Samuel Frank; Claudia M Testa; David Stamler; Elise Kayson; Charles Davis; Mary C Edmondson; Shari Kinel; Blair Leavitt; David Oakes; Christine O'Neill; Christina Vaughan; Jody Goldstein; Margaret Herzog; Victoria Snively; Jacquelyn Whaley; Cynthia Wong; Greg Suter; Joseph Jankovic; Joohi Jimenez-Shahed; Christine Hunter; Daniel O Claassen; Olivia C Roman; Victor Sung; Jenna Smith; Sarah Janicki; Ronda Clouse; Marie Saint-Hilaire; Anna Hohler; Denyse Turpin; Raymond C James; Ramon Rodriguez; Kyle Rizer; Karen E Anderson; Hope Heller; Alexis Carlson; Susan Criswell; Brad A Racette; Fredy J Revilla; Frederick Nucifora; Russell L Margolis; MaryJane Ong; Tilak Mendis; Neila Mendis; Carlos Singer; Monica Quesada; Jane S Paulsen; Thomas Brashers-Krug; Amanda Miller; Jane Kerr; Richard M Dubinsky; Carolyn Gray; Stewart A Factor; Elaine Sperin; Eric Molho; Mary Eglow; Sharon Evans; Rajeev Kumar; Christina Reeves; Ali Samii; Sylvain Chouinard; Monica Beland; Burton L Scott; Patrick T Hickey; Sherali Esmail; Wai Lun Alan Fung; Clare Gibbons; Lina Qi; Amy Colcher; Cory Hackmyer; Andrew McGarry; Kevin Klos; Mark Gudesblatt; Lori Fafard; Laura Graffitti; Daniel P Schneider; Rohit Dhall; Joanne M Wojcieszek; Kathrin LaFaver; Andrew Duker; Erin Neefus; Hilary Wilson-Perez; David Shprecher; Paola Wall; Karen A Blindauer; Lynn Wheeler; James T Boyd; Emily Houston; Eric S Farbman; Pinky Agarwal; Shirley W Eberly; Arthur Watts; Pierre N Tariot; Andrew Feigin; Scott Evans; Chris Beck; Constance Orme; Jon Edicola; Emily Christopher Journal: JAMA Date: 2016-07-05 Impact factor: 56.272
Authors: N E Carlozzi; S G Schilling; J-S Lai; J S Paulsen; E A Hahn; J S Perlmutter; C A Ross; N R Downing; A L Kratz; M K McCormack; M A Nance; K A Quaid; J C Stout; R C Gershon; R E Ready; J A Miner; S K Barton; S L Perlman; S M Rao; S Frank; I Shoulson; H Marin; M D Geschwind; P Dayalu; S M Goodnight; D Cella Journal: Qual Life Res Date: 2016-08-13 Impact factor: 4.147
Authors: N E Carlozzi; S G Schilling; J-S Lai; J S Perlmutter; M A Nance; J F Waljee; J A Miner; S K Barton; S M Goodnight; P Dayalu Journal: Qual Life Res Date: 2016-04-01 Impact factor: 4.147