title: The Key Search Subtest of the Behavioural Assessment of the Dysexecutive Syndrome in Children (BADS-C) Instrument Reveals Impaired Planning Without External Constraints in Children With Neurofibromatosis Type 1.
abstract: Studies of executive function and its relationship with brain T2-weighted hyperintensities in children with neurofibromatosis type 1 (NF1) have yielded inconsistent results. We examined 16 children with NF1 aged 8 to 15 years, of normal intelligence, and compared their findings to those of 16 siblings and 16 typically developing children using the Behavioural Assessment of the Dysexecutive Syndrome in Children (BADS-C). NF1 patients had an adequate overall score at BADS-C, but showed significantly lower performance than typical peers in the Key Search subtest. This is a task that must be solved without any given rules, in which subjects must devise a strategy and an efficient search pattern transferable to other similar real situations. The Key Search scores were not correlated with number and signal characteristics of T2-weighted hyperintensities. Planning without external indications is impaired in children with NF1 because they have to rely entirely on self-organization and monitoring; this study provides information for remediation programs designed to improve functioning in daily life.
authors.affiliation: 1 Developmental Neurology Division, Fondazione IRCCS Istituto Neurologico C. Besta, Milan, Italy.
authors.forename: Daria
authors.initials: D
authors.lastname: Riva
authors.affiliation: 1 Developmental Neurology Division, Fondazione IRCCS Istituto Neurologico C. Besta, Milan, Italy.
authors.forename: Chiara
authors.initials: C
authors.lastname: Vago
authors.affiliation: 2 Neuroradiology Department, Fondazione IRCCS Istituto Neurologico C. Besta, Milan, Italy.
authors.forename: Alessandra
authors.initials: A
authors.lastname: Erbetta
authors.affiliation: 1 Developmental Neurology Division, Fondazione IRCCS Istituto Neurologico C. Besta, Milan, Italy.
authors.forename: Veronica
authors.initials: V
authors.lastname: Saletti
authors.affiliation: 1 Developmental Neurology Division, Fondazione IRCCS Istituto Neurologico C. Besta, Milan, Italy.
authors.forename: Silvia
authors.initials: S
authors.lastname: Esposito
authors.affiliation: 3 Pediatric Neuropsychiatry, Spedali Civili of Brescia, Brescia, Italy.
authors.forename: Roberto
authors.initials: R
authors.lastname: Micheli
authors.affiliation: 1 Developmental Neurology Division, Fondazione IRCCS Istituto Neurologico C. Besta, Milan, Italy.
authors.forename: Sara
authors.initials: S
authors.lastname: Bulgheroni
mesh_terms: D000293:Adolescent; D000704:Analysis of Variance; D001921:Brain / Q000000981:diagnostic imaging; D016022:Case-Control Studies; D002648:Child; D056344:Executive Function; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009456:Neurofibromatosis 1 / Q000175:diagnosis* / Q000523:psychology*; D009483:Neuropsychological Tests; D011446:Prospective Studies; D035781:Siblings; D013850:Thinking; D014888:Wechsler Scales
keywords: children; executive functions; magnetic resonance imaging (MRI) T2 hyperintensities; neurofibromatosis type 1; planning