Pilot Study of a Novel Computerized Task to Assess Spatial Learning in Children and Adolescents With Neurofibromatosis Type 1

Ullrich, Nicole J.; Ayr, Lauren K.; Leaffer, Emily B.; Irons, Mira; Rey‐Casserly, Celiane

doi:10.1177/0883073809358454

Cited by 32 publications

(31 citation statements)

References 38 publications

(57 reference statements)

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“…In the pilot study, we were able to demonstrate a correlation between measures of working memory/executive function and the Arena Maze variables. In the current study, however, we did not find a relationship between Arena Maze performance and other measures of visual spatial skills, visual learning, attention, or executive function . There was some improvement seen in latency on one trial over the course of the study for the whole group, but there was no evidence of treatment response.…”

Section: Discussioncontrasting

confidence: 91%

“…Since the standard tests used to measure patient spatial learning and memory in humans differ from the Morris Water Maze used in animal models, the Arena Maze was developed to assess spatial learning strategies in young adults following traumatic brain injury and in a pilot study among children with NF1 and their unaffected siblings . The objective of the current study was to evaluate the feasibility of the Arena Maze to assess spatial learning in children with NF1.…”

Section: Introductionmentioning

confidence: 99%

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Visual spatial learning outcomes for clinical trials in neurofibromatosis type 1

Ullrich

Payne

Walsh

et al. 2020

Ann Clin Transl Neurol

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Cognitive problems are common in children with neurofibromatosis type 1, representing a significant source of lifelong morbidity. Assessment of cognitive function has been challenging in the setting of clinical trials. Spatial learning deficits may be an important target for cognitive interventions. We leveraged a large, international cognitive study in affected children with NF1 treated with lovastatin to assess spatial learning using the “Arena Maze”, a portable, computerized task that allows for retesting in the same environment. As with the parent study, spatial learning assessed with this task did not improve with lovastatin treatment.

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Section: Discussioncontrasting

confidence: 91%

Section: Introductionmentioning

confidence: 99%

Visual spatial learning outcomes for clinical trials in neurofibromatosis type 1

Ullrich

Payne

Walsh

et al. 2020

Ann Clin Transl Neurol

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“…found no significant impairment on the 15 words of Rey (immediate or delayed recall). Ullrich et al 40 . found no differences between NF1 and comparison groups on the California Verbal Learning Test for Children.…”

Section: Resultsmentioning

confidence: 95%

Behaviour in children with neurofibromatosis type 1: cognition, executive function, attention, emotion, and social competence

Lehtonen

Howie

Trump

et al. 2012

Develop Med Child Neuro

183

177

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AIM This systematic review aimed to pull together the findings from research into behavioural systems and attention in children with neurofibromatosis type 1 (NF1) and to identify areas that need further study.METHOD Relevant papers were identified through searches of electronic databases (MEDLINE, PsycINFO, EMBASE) and manual searches through reference lists. In total, 5746 articles were identified and 57 met the inclusion criteria. The data were synthesized using the narrative approach, as the studies varied considerably in terms of participants and measures. RESULTSThe results of the review showed that intelligence, academic skills, visuospatial skills, social competence, and attention are impaired in children with NF1. Evidence of deficits in memory, motor functioning, language, and executive functions was less clear. INTERPRETATIONResearch has made marked progress in outlining the behavioural phenotype of NF1. However, although the general areas of impairment are becoming better known, the exact nature of the impairment is still not understood in many areas of behaviour. Care needs to be taken with the way in which behavioural constructs are defined and measured, and the variability of problems in NF1 is a particular challenge. Nevertheless, research is steadily moving towards comprehensive understanding of behaviour in children with NF1.Neurofibromatosis type 1 (NF1) is one of the most common genetic disorders with a prevalence in the UK of 1 per 4560 live births. 1 The main disease feature in childhood is the emergence of multiple café-au-lait spots (flat coffee-coloured skin lesions) and skinfold freckling. The most common complications in childhood are different deficits of cognition and behaviour, which affect the majority of children with NF1. Children with NF1 are also at a higher risk of a number of much rarer physical complications that can affect virtually every system of the body 2 and include disfiguring plexiform neurofibromas, scoliosis, pseudarthrosis, and optic nerve gliomas.The cognitive and behavioural problems associated with NF1 are a major impairment to children's functioning at school and are issues that repeatedly come up in clinical practice as causing great distress to parents. The IQ of children with NF1 is usually within the normal range although somewhat lower than that of their unaffected siblings or community comparisons. More than 70% of children with NF1 perform more poorly at school than would be expected given their intellectual abilities.3 Furthermore, only a small proportion of children with NF1 are diagnosed with attention-deficit-hyperactivity disorder (ADHD), 4 although experimental studies using clinical diagnostic criteria for ADHD suggest that the incidence of this disorder among children with NF1 is up to 40 to 50%. 3 Issues concerning learning and various behaviour problems (such as difficulties with attention, peer relationships, and challenging behaviours) are among the most common complaints by parents of children with NF1 that emerge in our clinic work. Questi...

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“…13 Additionally, behavioural deficits in patients with neurofibromatosis type 1 and mice are very similar: most notably in their analogous deficits in (virtual) watermaze performance. 11,29,30 Mechanistically, GABAergic dysfunction has been observed in both the mouse model and patients. 11,12,31 Nevertheless, in view of the results of our trial, further insight into the pathophysiology of neurofibromatosis type 1 will be necessary to explore other targetable disease mechanisms.…”

Section: Discussionmentioning

confidence: 99%

Simvastatin for cognitive deficits and behavioural problems in patients with neurofibromatosis type 1 (NF1-SIMCODA): a randomised, placebo-controlled trial

Vaart

Plasschaert

Rietman

et al. 2013

The Lancet Neurology

113

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Pilot Study of a Novel Computerized Task to Assess Spatial Learning in Children and Adolescents With Neurofibromatosis Type 1

Cited by 32 publications

References 38 publications

Visual spatial learning outcomes for clinical trials in neurofibromatosis type 1

Visual spatial learning outcomes for clinical trials in neurofibromatosis type 1

Behaviour in children with neurofibromatosis type 1: cognition, executive function, attention, emotion, and social competence

Simvastatin for cognitive deficits and behavioural problems in patients with neurofibromatosis type 1 (NF1-SIMCODA): a randomised, placebo-controlled trial

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