Cognitive Profile of Neurofibromatosis Type 1

Levine, Terry M.; Materek, April; Abel, Jessica; O’Donnell, Madeline; Cutting, Laurie E.

doi:10.1016/j.spen.2006.01.006

Cited by 130 publications

(146 citation statements)

References 34 publications

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“…33, 34 The effect of age exceeds the effect of radiation dose. This provides guidance regarding selection of patients for treatment, and it additionally demonstrates the overarching influence of age and increasing deficits with time in younger patients.…”

Section: Discussionmentioning

confidence: 99%

Late Effects of Conformal Radiation Therapy for Pediatric Patients With Low-Grade Glioma: Prospective Evaluation of Cognitive, Endocrine, and Hearing Deficits

Merchant

Conklin

et al. 2009

JCO

356

249

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A B S T R A C T PurposeWe conducted a prospective trial to evaluate late effects in pediatric patients with low-grade glioma (LGG) treated with conformal radiation therapy (CRT). Patients and MethodsBetween August 1997 and August 2006, 78 pediatric patients with LGG (mean age, 9.7 years; standard deviation, Ϯ4.4 years) received 54 Gy of CRT with a 10-mm clinical target volume margin. Tumor locations were diencephalon (n ϭ 58), cerebral hemisphere (n ϭ 3), and cerebellum (n ϭ 17). Baseline and serial evaluations were performed to identify deficits in cognition, endocrine function, and hearing. Deficits were correlated with clinical factors and radiation dose within specific normal tissue volumes. ResultsCognitive effects of CRT through 5 years after CRT correlated with patient age, neurofibromatosis type 1 status, tumor location and volume, extent of resection, and radiation dose. The effect of age exceeded that of radiation dose; patients younger than 5 years experienced the greatest decline in cognition. Before CRT, growth hormone (GH) secretion abnormality was diagnosed in 24% of tested patients, and 12% had precocious puberty. The 10-year cumulative incidence of GH replacement was 48.9%; of thyroid hormone replacement, 64.0%; of glucocorticoid replacement, 19.2%; and of gonadotropin-releasing hormone analog therapy, 34.2%. The mean Ϯ standard errors of the cumulative incidence of hearing loss at 10 years did not exceed 5.7% Ϯ 3.3% at any frequency. ConclusionTo our knowledge, this is the largest series of prospectively followed children with LGG to undergo irradiation. Adverse effects are limited and predictable for most patients; however, this study provides additional evidence that CRT should be delayed for young patients and identifies the potential benefits of reducing radiation dose to normal brain.

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Section: Discussionmentioning

confidence: 99%

Late Effects of Conformal Radiation Therapy for Pediatric Patients With Low-Grade Glioma: Prospective Evaluation of Cognitive, Endocrine, and Hearing Deficits

Merchant

Conklin

et al. 2009

JCO

356

249

View full text Add to dashboard Cite

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“…In keeping with previous findings [8,9], it was hypothesised that, overall, children with NF1 would demonstrate poorer social competence compared with their typically developing peers (Hypothesis 1). However, given the variability observed in the clinical phenotype of children with NF1 [43,45], significant variability in their social competence was also anticipated (Hypothesis 2).…”

Section: Aims Of the Current Studymentioning

confidence: 96%

Social Competence in Children with Neurofibromatosis Type 1: Relationships with Psychopathology and Cognitive Ability

Lewis¹,

Porter

Williams³

et al. 2016

IPCDD

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“…Investigations of the neuropsychological profiles of children with NF1 have consistently documented a general "downward shift" in overall IQ (Ferner et al 1996), with the mean intellectual functioning of children with NF1 in the low end of the average to low average range (Levine et al 2006;North 1998North , 2000. Beyond intellect, studies have found specific deficits in visual perception, most consistently on the Benton Judgment of Line Orientation (JLO) task (Rowbotham et al 2009;Hyman et al 2005;Schrimsher et al 2003;Benton et al 1983), along with more generalized difficulties in the areas of language development (Hyman et al 2005), executive functioning (Templer et al 2012;Roy et al 2010;North et al 2002), and attention (Templer et al 2012;North et al 2002).…”

mentioning

confidence: 99%

The Relation Between ADHD and Cognitive Profiles of Children with NF1

Potvin

Hardy

Walsh

2015

J Pediatr Neuropsychol

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Despite well-accepted findings of a "downward shift" in intellectual functioning in children with neurofibromatosis type 1 (NF1), research has not examined the contribution of the individual indices in this lower performance on intelligence (IQ) measures. Although 30-50 % of children with NF1 meet criteria for a diagnosis of attention deficit/ hyperactivity disorder (ADHD), research has not established the relation between ADHD and intellectual profiles in these children. To clarify these issues, this study examined the Wechsler Intelligence Scale for Children, Fourth Edition (WISC-IV) profiles of children with NF1, with and without comorbid ADHD, using a comparison group of children with developmental ADHD. We found that rather than an overall lowering of IQ, children with NF1 demonstrated an uneven intellectual profile. While verbal abilities were generally preserved, nonverbal, working memory, and processing speed abilities were lower than the population norms. Children with NF1 and comorbid ADHD showed significantly lower performance on tests of working memory than children with NF1 without ADHD or children with developmental ADHD. These results suggest that global intellect is inadequate in describing the cognitive phenotype in children with NF1 and that in children with NF1 comorbid ADHD may manifest as additional cognitive burden with respect to working memory.

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Cognitive Profile of Neurofibromatosis Type 1

Cited by 130 publications

References 34 publications

Late Effects of Conformal Radiation Therapy for Pediatric Patients With Low-Grade Glioma: Prospective Evaluation of Cognitive, Endocrine, and Hearing Deficits

Late Effects of Conformal Radiation Therapy for Pediatric Patients With Low-Grade Glioma: Prospective Evaluation of Cognitive, Endocrine, and Hearing Deficits

Social Competence in Children with Neurofibromatosis Type 1: Relationships with Psychopathology and Cognitive Ability

The Relation Between ADHD and Cognitive Profiles of Children with NF1

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