Remote Assessment of Cognitive Function in Juvenile Neuronal Ceroid Lipofuscinosis (Batten disease): A Pilot Study of Feasibility and Reliability. (March 2016)

• Record Type: Journal Article
• Authors: Ragbeer, Shayne N.; Augustine, Erika F.; Mink, Jonathan W.; Thatcher, Alyssa R.; Vierhile, Amy E.; Adams, Heather R.
• Is Part Of: Journal of child neurology. Volume 31:Number 4(2016:Apr.)
• Page Start: 481
• Page End: 487
• Publication Date: 2016-03
• Subjects: Batten disease -- CLN3 -- JNCL -- telemedicine -- neurodegenerative disease; Nervous system -- Diseases -- Periodicals; 618.928
• DOI: 10.1177/0883073815600863
• Languages: English
• ISSNs: 0883-0738

Abstract

Remote technology provides an opportunity to extend the reach of clinical care and research for pediatric rare disease. This pilot study evaluated the feasibility and reliability of neuropsychological evaluation, using remote audiovisual technology, in the assessment of children with juvenile Batten disease. Three children with Batten disease and 1 healthy sibling completed a standardized cognitive assessment. Results indicated high agreement between an in-person and a remote evaluator when comparing the subjects' cognitive test scores. This initial test of remote cognitive assessment suggests it is feasible and reliable in children with pediatric neurodegenerative disease, for whom disease burden may limit travel and access to expert care and/or clinical trials.