19
views
0
recommends
+1 Recommend
0 collections
    0
    shares
      • Record: found
      • Abstract: found
      • Article: found
      Is Open Access

      Cognitive and Behavioral Disorders in Children with Neurofibromatosis Type 1

      review-article

      Read this article at

      Bookmark
          There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

          Abstract

          Aim

          The last systematic review of research on the behavior of children with neurofibromatosis type 1 (NF1) was in 2012. Since then, several important findings have been published. Therefore, the study aim was to synthesize recent relevant work related to this issue.

          Method

          We conducted a systematic review of the literature. Relevant articles were identified using the electronic databases PubMed, PsycINFO, and Scopus and a manual search of references lists. Thirty of 156 articles identified met the inclusion criteria. A quality evaluation of the articles was performed and the information was synthesized using a narrative approach.

          Results

          Compared with controls, children and adolescents with NF1 present significant alterations in language, reading, visuospatial skills, motor function, executive function, attention, behavior, emotion, and social skills. The prevalence of attention-deficit/hyperactivity disorder (ADHD) is important and can affect cognition and executive function variables. A high prevalence of autistic traits and autistic spectrum disorder were reported. The benefits of using statins to treat cognitive deficits are unclear. However, children with NF1 and ADHD seem to benefit from methylphenidate treatment. The presence of hyperintensities in brain magnetic resonance imaging data seem to be related to poor cognitive performance. Analysis of these lesions could help to predict cognitive alterations in children with NF1.

          Interpretation

          There has been important progress to evaluate cognitive characteristics of children with NF1 and to determine the physiological mechanisms of the concomitant disorders. However, discrepancies in relation to intelligence, learning disabilities, attention deficits, and treatment remain. Further investigations on this topic are recommended.

          Related collections

          Most cited references73

          • Record: found
          • Abstract: not found
          • Article: not found

          Preferred reporting items for systematic reviews and meta-analyses: the PRISMA statement.

            Bookmark
            • Record: found
            • Abstract: found
            • Article: not found

            Why IQ is not a covariate in cognitive studies of neurodevelopmental disorders.

            IQ scores are volatile indices of global functional outcome, the final common path of an individual's genes, biology, cognition, education, and experiences. In studying neurocognitive outcomes in children with neurodevelopmental disorders, it is commonly assumed that IQ can and should be partialed out of statistical relations or used as a covariate for specific measures of cognitive outcome. We propose that it is misguided and generally unjustified to attempt to control for IQ differences by matching procedures or, more commonly, by using IQ scores as covariates. We offer logical, statistical, and methodological arguments, with examples from three neurodevelopmental disorders (spina bifida meningomyelocele, learning disabilities, and attention deficit hyperactivity disorder) that: (1) a historical reification of general intelligence, g, as a causal construct that measures aptitude and potential rather than achievement and performance has fostered the idea that IQ has special status and that in studying neurocognitive function in neurodevelopmental disorders; (2) IQ does not meet the requirements for a covariate; and (3) using IQ as a matching variable or covariate has produced overcorrected, anomalous, and counterintuitive findings about neurocognitive function.
              Bookmark
              • Record: found
              • Abstract: found
              • Article: not found

              Use of the national institutes of health criteria for diagnosis of neurofibromatosis 1 in children.

              The National Institutes of Health (NIH) Diagnostic Criteria for neurofibromatosis 1 (NF1) are very useful clinically, but some individuals who are later shown to have NF1 cannot be diagnosed in early childhood using these criteria. The aim of this study is to determine the value of the NIH Diagnostic Criteria for NF1 in early childhood, to determine the age at which diagnosis can confidently be made, and to clarify the age at onset of the cardinal clinical features used in the NIH Diagnostic Criteria. We studied 1893 NF1 patients under 21 years old from the National Neurofibromatosis Foundation International Database to determine the age at which the features included in the NIH Diagnostic Criteria appear. Approximately 46% of sporadic NF1 cases fail to meet the NIH Diagnostic Criteria by 1 year of age. Nearly all (97%; 95% confidence interval: 94-98) NF1 patients meet the criteria for diagnosis by 8 years old, and all do so by 20 years old. The usual order of appearance of the clinical features listed as NIH criteria is café-au-lait macules, axillary freckling, Lisch nodules, and neurofibromas. Symptomatic optic glioma is usually diagnosed by 3 years old, and characteristic osseous lesions are usually apparent within the first year of life. The diagnosis of NF1 cannot always be made in young children using the NIH Diagnostic Criteria. Modification of these criteria may be necessary for children under 8 years old.
                Bookmark

                Author and article information

                Contributors
                URI : http://frontiersin.org/people/u/436183
                URI : http://frontiersin.org/people/u/152391
                URI : http://frontiersin.org/people/u/123503
                Journal
                Front Pediatr
                Front Pediatr
                Front. Pediatr.
                Frontiers in Pediatrics
                Frontiers Media S.A.
                2296-2360
                30 October 2017
                2017
                : 5
                : 227
                Affiliations
                [1] 1Neurosciences Research Group, Medicine and Health Sciences School, Universidad del Rosario , Bogota, Colombia
                Author notes

                Edited by: Carl E. Stafstrom, University of Wisconsin-Madison, United States

                Reviewed by: D. Mishra, Maulana Azad Medical College, India; Brahim Tabarki, University of Sousse, Tunisia

                *Correspondence: Alberto Velez Van Meerbeke, alberto.velez@ 123456urosario.edu.co

                Specialty section: This article was submitted to Neuropediatrics, a section of the journal Frontiers in Pediatrics

                Article
                10.3389/fped.2017.00227
                5670111
                29164079
                edf3dfbe-302d-4fb3-a1b5-5d74e7f2c51a
                Copyright © 2017 Torres Nupan, Velez Van Meerbeke, López Cabra and Herrera Gomez.

                This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

                History
                : 06 June 2017
                : 05 October 2017
                Page count
                Figures: 1, Tables: 6, Equations: 0, References: 76, Pages: 18, Words: 14099
                Categories
                Pediatrics
                Review

                neurofibromatosis,neurofibromatosis type 1,cognitive functioning,behavior,attention-deficit/hyperactivity disorder,autism spectrum disorder,executive functions,visuospatial functioning

                Comments

                Comment on this article