OBJECTIVE: To determine if remote administration of the Unified Batten Disease Rating Scale (UBDRS) Physical Impairment subscale by telemedicine is reliable and feasible across a broad range of disease severity. METHODS: For the majority (n = 10) of subjects, the examination was performed by a nonphysician who had been trained to perform the examination but not to score the subjects. A trained rater scored the subjects via live video; a second trained rater performed a separate examination in person and scored that examination. For 3 telemedicine evaluations, examinations were performed and scored by a trained rater while a second trained rater simultaneously scored the subjects via live video. Reliability was determined by intraclass correlation coefficient (ICC). RESULTS: Subjects (n = 13) represented a wide range of disease severity. Remote administration of the UBDRS Physical Impairment subscale had high interrater reliability across all subjects (ICC = 0.94). When only the subjects (n = 10) who had been examined by the nonphysician and scored remotely were included in the analysis, the reliability was unchanged (ICC = 0.95). CONCLUSIONS: The UBDRS Physical Impairment subscale is reliable and feasible for remote administration. Telemedicine has the potential to be a useful tool in rare neurologic disease research and clinical assessment.
OBJECTIVE: To determine if remote administration of the Unified Batten Disease Rating Scale (UBDRS) Physical Impairment subscale by telemedicine is reliable and feasible across a broad range of disease severity. METHODS: For the majority (n = 10) of subjects, the examination was performed by a nonphysician who had been trained to perform the examination but not to score the subjects. A trained rater scored the subjects via live video; a second trained rater performed a separate examination in person and scored that examination. For 3 telemedicine evaluations, examinations were performed and scored by a trained rater while a second trained rater simultaneously scored the subjects via live video. Reliability was determined by intraclass correlation coefficient (ICC). RESULTS: Subjects (n = 13) represented a wide range of disease severity. Remote administration of the UBDRS Physical Impairment subscale had high interrater reliability across all subjects (ICC = 0.94). When only the subjects (n = 10) who had been examined by the nonphysician and scored remotely were included in the analysis, the reliability was unchanged (ICC = 0.95). CONCLUSIONS: The UBDRS Physical Impairment subscale is reliable and feasible for remote administration. Telemedicine has the potential to be a useful tool in rare neurologic disease research and clinical assessment.
Authors: E Ray Dorsey; Lisa M Deuel; Tiffini S Voss; Kara Finnigan; Benjamin P George; Sheelah Eason; David Miller; Jason I Reminick; Anna Appler; Joyce Polanowicz; Lucy Viti; Sandy Smith; Anthony Joseph; Kevin M Biglan Journal: Mov Disord Date: 2010-08-15 Impact factor: 10.338
Authors: J M Kwon; H Adams; P G Rothberg; E F Augustine; F J Marshall; E A Deblieck; A Vierhile; C A Beck; N J Newhouse; J Cialone; E Levy; D Ramirez-Montealegre; L S Dure; K R Rose; J W Mink Journal: Neurology Date: 2011-10-19 Impact factor: 9.910
Authors: F J Marshall; E A de Blieck; J W Mink; L Dure; H Adams; S Messing; P G Rothberg; E Levy; T McDonough; J DeYoung; M Wang; D Ramirez-Montealegre; J M Kwon; D A Pearce Journal: Neurology Date: 2005-07-26 Impact factor: 9.910
Authors: Heather R Adams; Jennifer Kwon; Frederick J Marshall; Elisabeth A de Blieck; David A Pearce; Jonathan W Mink Journal: J Child Neurol Date: 2007-05 Impact factor: 1.987
Authors: H Ben-Pazi; P Browne; P Chan; E Cubo; M Guttman; A Hassan; J Hatcher-Martin; Z Mari; E Moukheiber; N U Okubadejo; A Shalash Journal: Curr Neurol Neurosci Rep Date: 2018-04-13 Impact factor: 5.081
Authors: Elisabeth A de Blieck; Erika F Augustine; Frederick J Marshall; Heather Adams; Jennifer Cialone; Leon Dure; Jennifer M Kwon; Nicole Newhouse; Katherine Rose; Paul G Rothberg; Amy Vierhile; Jonathan W Mink Journal: Contemp Clin Trials Date: 2013-04-26 Impact factor: 2.226
Authors: Elsa Shapiro; Jessica Bernstein; Heather R Adams; Ann J Barbier; Teresa Buracchio; Peter Como; Kathleen A Delaney; Florian Eichler; Jonathan C Goldsmith; Melissa Hogan; Sarrit Kovacs; Jonathan W Mink; Joanne Odenkirchen; Melissa A Parisi; Alison Skrinar; Susan E Waisbren; Andrew E Mulberg Journal: Mol Genet Metab Date: 2016-04-14 Impact factor: 4.797
Authors: Shayne N Ragbeer; Erika F Augustine; Jonathan W Mink; Alyssa R Thatcher; Amy E Vierhile; Heather R Adams Journal: J Child Neurol Date: 2015-09-02 Impact factor: 1.987
Authors: Tyler B Johnson; Jacob T Cain; Katherine A White; Denia Ramirez-Montealegre; David A Pearce; Jill M Weimer Journal: Nat Rev Neurol Date: 2019-03 Impact factor: 42.937
Authors: Christopher G Tarolli; Grace A Zimmerman; Steven Goldenthal; Blake Feldman; Sarah Berk; Bernadette Siddiqi; Catherine M Kopil; Sohini Chowdhury; Kevin M Biglan; E Ray Dorsey; Jamie L Adams Journal: Neurol Clin Pract Date: 2020-02