title: Matching pairs difficulty in children with spinal muscular atrophy type I.
abstract: This study aimed to investigate the performance on pair-matching tasks in children with Spinal Muscular Atrophy type I (SMA-I) and the relationship between this performance and motor function, functional independence and quality of life. SMA-I (n = 12; 6.0 ± 2.3 yrs; 9 boys, 3 girls) and control sex-, age-matched children (n = 12; 6.2 ± 2.6 yrs) performed four pair-matching figure, number and letter tasks. The eye tracker detected eye movements. SMA-I children were assessed with CHOP INTEND, Pediatric Evaluation of Disability Inventory, and Pediatric Quality of Life Inventory. Analysis of variance showed that SMA-I children had a lower percentage of correct answers and longer timed performance compared to controls (p < 0.05). Pediatric Evaluation of Disability Inventory score (social function domain) was correlated to the percentage of correct answers on the pair-matching tasks on task 1 (r = 0.81; p = 0.001) and task 2 (r = 0.66; p = 0.020). Pair-matching performance of SMA-I children was poorer than the performance of control children. There was a relationship between pair-matching performance and social function. The restricted interaction with the environment, due to severe paralysis and poor verbal communication, is associated with cognitive difficulties in SMA-I children. The eye tracker was helpful in cognitive assessment of SMA-I children, who responded to the cognitive tests with eye movements.
authors.affiliation: Department of Physical Therapy, Speech Therapy and Occupational Therapy, Faculty of Medicine, University of São Paulo, São Paulo, SP, Brazil.
authors.forename: Graziela Jorge
authors.initials: GJ
authors.lastname: Polido
authors.affiliation: Department of Physical Therapy, Speech Therapy and Occupational Therapy, Faculty of Medicine, University of São Paulo, São Paulo, SP, Brazil.
authors.forename: Alessandra Ferreira
authors.initials: AF
authors.lastname: Barbosa
authors.affiliation: Computer Science Department, Institute of Mathematics and Statistics, University of São Paulo, São Paulo, SP, Brazil.
authors.forename: Carlos Hitoshi
authors.initials: CH
authors.lastname: Morimoto
authors.affiliation: Department of Physical Therapy, Speech Therapy and Occupational Therapy, Faculty of Medicine, University of São Paulo, São Paulo, SP, Brazil.
authors.forename: Fátima Aparecida
authors.initials: FA
authors.lastname: Caromano
authors.affiliation: Department of Neurology, UNIFESP (Federal University of São Paulo), São Paulo, SP, Brazil.
authors.forename: Francis Meire
authors.initials: FM
authors.lastname: Favero
authors.affiliation: Department of Neurology, Medical School of the University of São Paulo, University of São Paulo, São Paulo, SP, Brazil.
authors.forename: Edmar
authors.initials: E
authors.lastname: Zanoteli
authors.affiliation: Department of Neurology, Medical School of the University of São Paulo, University of São Paulo, São Paulo, SP, Brazil.
authors.forename: Umbertina Conti
authors.initials: UC
authors.lastname: Reed
authors.affiliation: Department of Physical Therapy, Speech Therapy and Occupational Therapy, Faculty of Medicine, University of São Paulo, São Paulo, SP, Brazil. Electronic address: ftmarivoos@gmail.com.
authors.forename: Mariana Callil
authors.initials: MC
authors.lastname: Voos
mesh_terms: D000704:Analysis of Variance; D002648:Child; D002675:Child, Preschool; D003071:Cognition; D004185:Disability Evaluation; D019988:Children with Disabilities / Q000523:psychology; D053483:Eye Movement Measurements; D005133:Eye Movements; D005260:Female; D006801:Humans; D008297:Male; D009483:Neuropsychological Tests; D010364:Pattern Recognition, Visual; D014897:Spinal Muscular Atrophies of Childhood / Q000235:genetics / Q000523:psychology*
keywords: Cognition; Spinal muscular atrophy; Visual perception