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Cognitive and Behavioral Disorders in Children with Neurofibromatosis Type 1

Overview
Journal Front Pediatr
Specialty Pediatrics
Date 2017 Nov 23
PMID 29164079
Citations 43
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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.

Citing Articles

Cognition and behavior in adults with neurofibromatosis type 1.

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Neurocognitive functioning in adults with neurofibromatosis type 1- a nationwide population-based study.

Doser K, Jepsen J, Kenborg L, Miskowiak K, Albieri V, Dalton S Orphanet J Rare Dis. 2024; 19(1):441.

PMID: 39609892 PMC: 11603635. DOI: 10.1186/s13023-024-03454-w.


[Progress and prospects in diagnosis and treatment of neurofibromatosis type 1].

Liu P Zhongguo Xiu Fu Chong Jian Wai Ke Za Zhi. 2024; 38(10):1166-1170.

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Autism Spectrum Disorder Symptom Profiles in Fragile X Syndrome, Angelman Syndrome, Tuberous Sclerosis Complex and Neurofibromatosis Type 1.

Lubbers K, Hiralal K, Dieleman G, Hagenaar D, Dierckx B, Legerstee J J Autism Dev Disord. 2024; .

PMID: 39395123 DOI: 10.1007/s10803-024-06557-2.


Lamotrigine for cognitive deficits associated with neurofibromatosis type 1: A phase II randomized placebo-controlled trial.

Ottenhoff M, Mous S, Castricum J, Rietman A, Oostenbrink R, van der Vaart T Dev Med Child Neurol. 2024; 67(4):537-549.

PMID: 39340758 PMC: 11875526. DOI: 10.1111/dmcn.16094.


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