Deborah A Hall1, Glenn T Stebbins1, Sebastien Jacquemont2, Elizabeth Berry-Kravis1,3, Christopher G Goetz1, Randi Hagerman4, Lin Zhang5, Maureen A Leehey6. 1. Department of Neurological Sciences and Pediatrics Rush University Chicago Illinois United States. 2. Department of Pediatrics University of Montreal Montreal Canada. 3. Department of Pediatrics Rush University Chicago Illinois United States. 4. MIND Institute and Department of Pediatrics University of California Davis School of Medicine Sacramento CA United States. 5. Department of Neurology University of California Davis School of Medicine Sacramento CA United States. 6. Department of Neurology University of Colorado School of Medicine Aurora Colorado United States.
Abstract
BACKGROUND: There are currently no proven treatments for fragile X-associated tremor and ataxia syndrome (FXTAS). Validated outcome measures are needed in order to plan and conduct clinical trials to aid in the development of therapy. METHODS: This study examined the reliability and construct validity of the FXTAS Rating Scale. The study was conducted by using ratings from movement disorder specialists, who were blinded to gene status, on the FXTAS Rating Scale. RESULTS: In 295 premutation carriers with and without FXTAS, 33 scale items showed a high level of overall reliability, adequate item-to-total correlations and construct validity. Factor analysis revealed four components. CONCLUSIONS: The result demonstrates that many items in the scale meet standard clinimetric criteria, but modification of the scale improved the overall utility.
BACKGROUND: There are currently no proven treatments for fragile X-associated tremor and ataxia syndrome (FXTAS). Validated outcome measures are needed in order to plan and conduct clinical trials to aid in the development of therapy. METHODS: This study examined the reliability and construct validity of the FXTAS Rating Scale. The study was conducted by using ratings from movement disorder specialists, who were blinded to gene status, on the FXTAS Rating Scale. RESULTS: In 295 premutation carriers with and without FXTAS, 33 scale items showed a high level of overall reliability, adequate item-to-total correlations and construct validity. Factor analysis revealed four components. CONCLUSIONS: The result demonstrates that many items in the scale meet standard clinimetric criteria, but modification of the scale improved the overall utility.
Authors: D A Hall; E Berry-Kravis; S Jacquemont; C D Rice; J Cogswell; L Zhang; R J Hagerman; P J Hagerman; M A Leehey Journal: Neurology Date: 2005-07-26 Impact factor: 9.910
Authors: James A Brunberg; Sebastien Jacquemont; Randi J Hagerman; Elizabeth M Berry-Kravis; Jim Grigsby; Maureen A Leehey; Flora Tassone; W Ted Brown; Claudia M Greco; Paul J Hagerman Journal: AJNR Am J Neuroradiol Date: 2002 Nov-Dec Impact factor: 3.825
Authors: P Trouillas; T Takayanagi; M Hallett; R D Currier; S H Subramony; K Wessel; A Bryer; H C Diener; S Massaquoi; C M Gomez; P Coutinho; M Ben Hamida; G Campanella; A Filla; L Schut; D Timann; J Honnorat; N Nighoghossian; B Manyam Journal: J Neurol Sci Date: 1997-02-12 Impact factor: 3.181
Authors: Maureen A Leehey; Elizabeth Berry-Kravis; Sung-Joon Min; Deborah A Hall; Cathlin D Rice; Lin Zhang; Jim Grigsby; Claudia M Greco; Ann Reynolds; Rebecca Lara; Jennifer Cogswell; Sebastien Jacquemont; David R Hessl; Flora Tassone; Randi Hagerman; Paul J Hagerman Journal: Mov Disord Date: 2007-01-15 Impact factor: 10.338
Authors: Sébastien Jacquemont; Randi J Hagerman; Maureen Leehey; Jim Grigsby; Lin Zhang; James A Brunberg; Claudia Greco; Vincent Des Portes; Tristan Jardini; Richard Levine; Elizabeth Berry-Kravis; W Ted Brown; Stephane Schaeffer; John Kissel; Flora Tassone; Paul J Hagerman Journal: Am J Hum Genet Date: 2003-03-12 Impact factor: 11.025
Authors: M A Leehey; E Berry-Kravis; C G Goetz; L Zhang; D A Hall; L Li; C D Rice; R Lara; J Cogswell; A Reynolds; L Gane; S Jacquemont; F Tassone; J Grigsby; R J Hagerman; P J Hagerman Journal: Neurology Date: 2007-12-05 Impact factor: 9.910
Authors: Joan A O'Keefe; Deborah Bang; Erin E Robertson; Alexandras Biskis; Bichun Ouyang; Yuanqing Liu; Gian Pal; Elizabeth Berry-Kravis; Deborah A Hall Journal: Mov Disord Clin Pract Date: 2020-08-29
Authors: Emily J Hill; Christopher G Goetz; Glenn T Stebbins; Randi Hagerman; Bichun Ouyang; Deborah A Hall Journal: Mov Disord Clin Pract Date: 2020-03-04
Authors: Nell Maltman; Janna Guilfoyle; Kritika Nayar; Gary E Martin; Molly Winston; Joseph C Y Lau; Lauren Bush; Shivani Patel; Michelle Lee; John Sideris; Deborah A Hall; Lili Zhou; Kevin Sharp; Elizabeth Berry-Kravis; Molly Losh Journal: Front Psychiatry Date: 2021-08-06 Impact factor: 5.435
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Authors: Erin E Robertson; Deborah A Hall; Gian Pal; Bichun Ouyang; Yuanqing Liu; Jessica M Joyce; Elizabeth Berry-Kravis; Joan A O'Keefe Journal: Clin Park Relat Disord Date: 2020-01-23
Authors: Diana A Abbasi; Thu T A Nguyen; Deborah A Hall; Erin Robertson-Dick; Elizabeth Berry-Kravis; Stephanie M Cologna Journal: Cerebellum Date: 2021-05-27 Impact factor: 3.847
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