Quantitative telemedicine ratings in Batten disease: implications for rare disease research.
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.
University of Rochester, Rochester, NY, USA.
J
J
Cialone
E F
EF
Augustine
N
N
Newhouse
A
A
Vierhile
F J
FJ
Marshall
J W
JW
Mink
D000293:Adolescent; D000328:Adult; D006801:Humans; D009472:Neuronal Ceroid-Lipofuscinoses / Q000175:diagnosis* / Q000503:physiopathology*; D009483:Neuropsychological Tests; D035583:Rare Diseases / Q000175:diagnosis; D015203:Reproducibility of Results; D012720:Severity of Illness Index; D017216:Telemedicine / Q000706:statistics & numerical data*; D014741:Video Recording / Q000706:statistics & numerical data; D057211:Webcasts as Topic / Q000706:statistics & numerical data; D055815:Young Adult