Neurocognitive profiles of learning disabled children with neurofibromatosis type 1

• Published in: Frontiers in human neuroscience Vol. 8; p. 386
• Main Authors: Orraca-Castillo, Miladys, Estévez-Pérez, Nancy, Reigosa-Crespo, Vivian
• Format: Journal Article
• Language: English
• Published: Switzerland Frontiers Research Foundation 06.06.2014; Frontiers Media S.A
• Subjects: Animal models; Brain; Children; Children & youth; Cognition; Cognition & reasoning; Cognitive ability; Congenital diseases; Developmental disabilities; developmental dyscalculia; developmental dyslexia; Disability; Dyslexia; Gender differences; Genetic disorders; Individual differences; Language; Learning disabilities; Mathematical models; Mathematics; Medical imaging; Memory; Neurodevelopmental disorders; Neurofibromatosis; neurofibromatosis type 1; Neurological disorders; Neuroscience; Neurosciences; Number systems; Phenotypes; phonological dyslexia; Phonology; Reading achievement; Reading comprehension; Recklinghausen's disease; Sex differences; specific learning disabilities; Studies; surface dyslexia; Tumors; Cuba; developmental dyscalculia; gender ratio; developmental dyslexia; neurofibromatosis type 1; specific learning disabilities; arithmetic dysfluency; surface dyslexia; phonological dyslexia
• ISSN: 1662-5161; 1662-5161
• DOI: 10.3389/fnhum.2014.00386

Neurofibromatosis 1 (NF1) is a genetic condition generally associated with intellectual deficiency and learning disabilities. Although there have been groundbreaking advances in the understanding of the molecular, cellular, and neural systems underlying learning deficits associated to NF1 in animal models, much remains to be learned about the spectrum of neurocognitive phenotype associated with the NF1 clinical syndrome. In the present study, 32 children with NF1 ranging from 7 to 14 years were evaluated with neurocognitive tests dedicated to assess basic capacities which are involved in reading and mathematical achievement. Deficits in lexical and phonological strategies and poor number facts retrieval were found underlying reading and arithmetic disorders, respectively. Additionally, efficiencies in lexical/phonological strategies and mental arithmetic were significant predictors of individual differences in reading attainment and math. However, deficits in core numeric capacities were not found in the sample, suggesting that it is not responsible for calculation dysfluency. The estimated prevalence of Developmental Dyscalculia was 18.8%, and the male:female ratio was 5:1. On the other hand, the prevalence of Developmental Dyslexia was almost 3 times as high (50%), and no gender differences were found (male: female ratio = 1:1). This study offers new evidence to the neurocognitive phenotype of NF1 contributing to an in depth understanding of this condition, but also to possible treatments for the cognitive deficits associated with NF1.