Remote Assessment of Cognitive Function in Juvenile Neuronal Ceroid Lipofuscinosis (Batten disease): A Pilot Study of Feasibility and Reliability.
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.
Department of Clinical and Social Sciences in Psychology, University of Rochester, Rochester, NY, USA.
Shayne N
SN
Ragbeer
Department of Neurology, School of Medicine and Dentistry, University of Rochester, Rochester, NY, USA.
Erika F
EF
Augustine
Department of Neurology, School of Medicine and Dentistry, University of Rochester, Rochester, NY, USA.
Jonathan W
JW
Mink
Department of Neurology, School of Medicine and Dentistry, University of Rochester, Rochester, NY, USA.
Alyssa R
AR
Thatcher
Department of Neurology, School of Medicine and Dentistry, University of Rochester, Rochester, NY, USA.
Amy E
AE
Vierhile
Department of Neurology, School of Medicine and Dentistry, University of Rochester, Rochester, NY, USA heather_adams@urmc.rochester.edu.
Heather R
HR
Adams
D000293:Adolescent; D002648:Child; D003071:Cognition; D005240:Feasibility Studies; D006801:Humans; D008297:Male; D008562:Membrane Glycoproteins / Q000235:genetics; D018832:Molecular Chaperones / Q000235:genetics; D009472:Neuronal Ceroid-Lipofuscinoses / Q000175:diagnosis* / Q000235:genetics / Q000523:psychology*; D009483:Neuropsychological Tests; D010865:Pilot Projects; D015203:Reproducibility of Results; D017216:Telemedicine / Q000379:methods*; D014195:Travel
C105199:CLN3 protein, human; D008562:Membrane Glycoproteins; D018832:Molecular Chaperones
Batten disease; CLN3; JNCL; neurodegenerative disease; telemedicine