Attention Skills in Children With Neurofibromatosis Type 1

Isenberg, Jill; Templer, Alexandra K.; Gao, Feng; Titus, Jeffrey B.; Gutmann, David H.

doi:10.1177/0883073812439435

Cited by 63 publications

(56 citation statements)

References 23 publications

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“…Emerging research suggests that problems with attention and related functions are likely to be one of the most common neuropsychological impairments in children with NS, as has been found to be the case in other RASopathies such as NF1 (e.g., Isenberg et al 2012;Payne et al 2011;Galasso et al 2014). Earlier studies using parent report measures such as the Child Behavior Checklist have noted children with NS showed greater difficulties related to attention and hyperactivity relative to comparison groups or normative data, although statistically significant differences were not always reported (van der Burgt et al 1999;Wood et al 1995;D.…”

Section: Attention and Executive Functioningmentioning

confidence: 90%

Neuropsychological Functioning in Individuals with Noonan Syndrome: a Systematic Literature Review with Educational and Treatment Recommendations

Pierpont

2015

J Pediatr Neuropsychol

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Noonan syndrome (NS) is a relatively common genetic syndrome with variable features including short stature, congenital heart disease, distinctive facial characteristics, skeletal anomalies, and varying degrees of developmental delay. NS is caused by gene mutations in a cellular signaling pathway that is essential for typical growth and development. Research in the past few decades has revealed that individuals with NS have highly variable neurocognitive and behavioral outcomes. To a certain extent, variability in cognitive functioning depends on the particular gene where a mutation is found; additionally, factors related to medical severity and environmental effects contribute substantially to outcomes. This article contains a systematic review of research on neuropsychological features of NS, including cognition, motor development, language, memory, attention, visual perception, adaptive behavior, social skills, and mental health concerns. Given the wide variety of possible cognitive and behavioral complications associated with NS, it is recommended that clinical neuropsychological evaluation of cognitive, adaptive, and psychological domains of functioning should be standard of care for all individuals with NS. Suggested considerations for assessment of individuals with NS are provided. The paper concludes with recommendations for educational and therapeutic interventions, as well as suggestions for future research directions.

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Section: Attention and Executive Functioningmentioning

confidence: 90%

Neuropsychological Functioning in Individuals with Noonan Syndrome: a Systematic Literature Review with Educational and Treatment Recommendations

Pierpont

2015

J Pediatr Neuropsychol

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“…As such, children and adults are prone to the development of benign and malignant tumors of the peripheral and central nervous systems. Within the CNS, children and adults affected with NF1 manifest cognitive disabilities, behavioral issues, and motor delays, which negatively impact on scholastic achievement (Hyman et al, 2005; 2006; Isenberg et al, 2012; Soucy et al, 2012). Moreover, 15–20% of children with NF1 will develop a low-grade glioma of the optic pathway, which when symptomatic can cause reduced vision (Listernick et al, 1994; 1997).…”

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confidence: 99%

Neurofibromatosis Type 1: Modeling CNS Dysfunction

et al. 2012

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Neurofibromatosis type 1 (NF1) is the most common monogenic disorder in which individuals manifest central nervous system (CNS) abnormalities. Affected individuals develop glial neoplasms (optic gliomas, malignant astrocytomas) and neuronal dysfunction (learning disabilities, attention deficits). Nf1 genetically-engineered mouse models have revealed the molecular and cellular underpinnings of gliomagenesis, attention deficit, and learning problems with relevance to basic neurobiology. Using NF1 as a model system, these studies have revealed critical roles for the NF1 gene in non-neoplastic cells in the tumor microenvironment, the importance of brain region heterogeneity, novel mechanisms of glial growth regulation, the neurochemical bases for attention deficit and learning abnormalities, and new insights into neural stem cell function. Here we review recent studies, presented at a symposium at the 2012 Society for Neuroscience annual meeting, that highlight unexpected cell biology insights into RAS and cyclic AMP pathway effects on neural progenitor signaling, neuronal function, and oligodendrocyte lineage differentiation.

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“…All primary outcomes capture high basal rates of impairment in NF1 11 12 33 58 59. Cognitive measures are analogous to MPH-responsive tests previously used in Nf1 mice,34–36 assessing sustained attention and spatial working memory.…”

Section: Methods and Analysismentioning

confidence: 99%

Effects of methylphenidate on cognition and behaviour in children with neurofibromatosis type 1: a study protocol for a randomised placebo-controlled crossover trial

et al. 2018

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IntroductionDopamine dysregulation has been identified as a key modulator of behavioural impairment in neurofibromatosis type 1 (NF1) and a potential therapeutic target. Preclinical research demonstrates reduced dopamine in the brains of genetically engineered NF1 mouse strains is associated with reduced spatial-learning and attentional dysfunction. Methylphenidate, a stimulant medication that increases dopaminergic and noradrenergic neurotransmission, rescued the behavioural and dopamine abnormalities. Although preliminary clinical trials have demonstrated that methylphenidate is effective in treating attention deficit hyperactivity disorder (ADHD) symptoms in children with NF1, its therapeutic effect on cognitive performance is unclear. The primary aim of this clinical trial is to assess the efficacy of methylphenidate for reducing attention deficits, spatial working memory impairments and ADHD symptoms in children with NF1.Methods and analysisA randomised, double-blind, placebo-controlled trial of methylphenidate with a two period crossover design. Thirty-six participants with NF1 aged 7–16 years will be randomised to one of two treatment sequences: 6 weeks of methylphenidate followed by 6 weeks of placebo or; 6 weeks of placebo followed by 6 weeks of methylphenidate. Neurocognitive and behavioural outcomes as well as neuroimaging measures will be completed at baseline and repeated at the end of each treatment condition (week 6, week 12). Primary outcome measures are omission errors on the Conners Continuous Performance Test-II (attention), between-search errors on the Spatial Working Memory task from the Cambridge Neuropsychological Test Automated Battery (spatial working memory) and the Inattentive and Hyperactivity/Impulsivity Symptom Scales on the Conners 3-Parent. Secondary outcomes will examine the effect of methylphenidate on executive functions, attention, visuospatial skills, behaviour, fine-motor skills, language, social skills and quality of life.Ethics and disseminationThis trial has hospital ethics approval and the results will be disseminated through peer-reviewed publications and international conferences.Trial registration numberACTRN12611000765921.

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Attention Skills in Children With Neurofibromatosis Type 1

Cited by 63 publications

References 23 publications

Neuropsychological Functioning in Individuals with Noonan Syndrome: a Systematic Literature Review with Educational and Treatment Recommendations

Neuropsychological Functioning in Individuals with Noonan Syndrome: a Systematic Literature Review with Educational and Treatment Recommendations

Neurofibromatosis Type 1: Modeling CNS Dysfunction

Effects of methylphenidate on cognition and behaviour in children with neurofibromatosis type 1: a study protocol for a randomised placebo-controlled crossover trial

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