Remote Assessment of Cognitive Function in Juvenile Neuronal Ceroid Lipofuscinosis (Batten disease)

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 one 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.

Agreement between telehealth and face-to-face assessment of intellectual ability in children with specific learning disorder

“…Previous studies used very small test batteries and often intentionally selected subtests that were predominantly verbal in nature (to be amenable to their technology/methodology). 21,22 The current study did not exclude nonverbal subtests even including a visuo-constructional task (block design); made possible by using two document cameras (one for the child and one for the assessor) and split-screen mechanisms (one showing the psychologist and the other presenting the block design). Furthermore, when visual tasks were attempted via telehealth in previous studies, the presentation of test material required the examiner to hold up the visual stimulus in front of the webcam or under a document camera.…”

“…Ragbeer et al (2015) conducted a feasibility pilot study involving three children with Batten disease and one healthy sibling (age range 10-16 years) on select subtests of the Wechsler Intelligence Scale for Children -Fourth Edition and the Wide Range Assessment of Memory and Learning -Second Edition. 21 Testing was undertaken in the same building in two separate rooms, with an assessor in one room and a graduate student sitting with the child in another. While the study clearly has limitations, such as exclusion of nonverbal subtests and small sample size, it nonetheless demonstrated initial feasibility as testing was well tolerated by children and the technology worked effectively.…”

“…Studies conducted have primarily involved adult populations and very small sample sizes. They have tended to look at specific populations 21 including rare conditions and those with lower intellectual abilities and predominantly involved verbal-based tasks that were purposefully selected to be more amenable to the telehealth technology available at the time. One study reported technical difficulties resulting in poor audio and visual quality.…”

Neurocognitive clinical outcome assessments for inborn errors of metabolism and other rare conditions

“…Remote assessments can reduce burdensome, costly travel for patients and families and increase patient access to research studies [13]. For researchers, remote assessment may facilitate data capture from a larger number of individuals and increase data quality by reducing the number of assessors, increasing measurement expertise, and providing a more controlled environment.…”

Remote Assessment of Cognition in Kids and Adolescents with Daytime Sleepiness: A pilot study of feasibility and reliability

“…In general, studies have also found participants are accepting and comfortable with the use of technology in performing cognitive assessments (Hildebrand et al, 2004; Parikh et al, 2013). Fewer pediatric studies exist assessing reliability of remote cognitive testing but include assessments of children with Batten disease, learning disabilities, and psychosis (Hodge et al, 2019; Ragbeer et al, 2016; Stain et al, 2011). However, none of the remote testing sites in these pediatric studies were conducted in home settings but rather hotel rooms during disease based conferences or within a designated testing site with patients in one room and assessor in another.…”