Quality of life and neurological disability in children and young people with ataxia telangiectasia

Abstract

AIM: To explore neurological factors affecting quality of life (QoL) in children and young people with ataxia-telangiectasia (A-T), from both child and parent perspective. METHOD: 24 children/young people with A-T (mean age 11.2 +/- 3.5 years; 13 males) and 20 parents were recruited, and 58% were reassessed after an average interval of 3.4 years. Participants completed the PedsQL QoL assessment. Participants with A-T underwent structured neurological examination. QoL data from 20 healthy controls and their parents was used for comparison. RESULTS: Children/young people with A-T rated their QoL higher than parental ratings across time points, with no longitudinal change. Higher age of the child participant correlated with lower parental (r = -0.43, p = .008) but not child ratings of QoL (r = -0.16, p = .380). Child and parent QoL ratings from the A-T group were lower than respective ratings from controls (etap2 = 0.44 and etap2 = 0.75 respectively, both p = 0.75 respectively, both p 2 = 0.44, p= = 0.44, p= Copyright © 2022 Published by Elsevier Ltd on behalf of European Paediatric Neurology Society.