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OBJECTIVE To assess the frequency and severity of specific cognitive deficits in children with neurofibromatosis type 1 (NF1) in a large unbiased cohort. METHODS Extensive cognitive assessments were performed in 81 children with NF1 ages 8 to 16 years and their performance was compared with that of 49 unaffected sibling controls. RESULTS Eighty-one(More)
Cognitive deficits are the most common complication in children with neurofibromatosis type 1 (NF1), and academic achievement is broadly affected. There is a lack of consensus in the literature regarding the frequency of general and specific learning disabilities, which seems to be related to the lack of a consensus on diagnostic criteria. The present study(More)
The aim of this study was to examine functional attention and executive deficits present in everyday living in a large sample of children with neurofibromatosis type 1 (NF1). Data are presented from 199 children with NF1 and 55 unaffected sibling controls who were administered the Behavior Rating Inventory of Executive Function (BRIEF) and Conners' ADHD(More)
Cognitive deficits and academic learning difficulties are the most common neurologic "complication" of neurofibromatosis 1 in childhood and can be responsible for significant lifetime morbidity. There is a slight increase in the frequency of mental retardation (Wechsler Full-Scale IQ < 70) in children with neurofibromatosis 1, but the mean Full-Scale IQ for(More)
BACKGROUND Neurofibromatosis type 1 (NF1) is a single gene disorder associated with a high frequency of cognitive deficits and a complex cognitive phenotype. These cognitive deficits have been associated with focal areas of high signal intensity on T2 weighted MRI images but the relationship remains controversial. METHOD A cohort of 76 children with NF1(More)
BACKGROUND Cognitive impairment is the most common complication of neurofibromatosis type 1 (NF1) in childhood. Current research suggests a strong relationship between cognitive deficits and brain T2-hyperintensities. The majority of these lesions disappear as the child ages. Cross-sectional data suggest that there also are improvements in intellect. (More)
Neurofibromatosis 1 is an autosomal dominant, multisystem disorder with myriad clinical manifestations. Between 1991 and 1998, 495 adults and children were diagnosed with neurofibromatosis 1 at a specialized neurogenetics clinic in Sydney, Australia. This review establishes the prevalence of the clinical manifestations of neurofibromatosis 1 in these(More)
Two children with Rett syndrome were treated for their self-injurious hand biting. Results of an operant assessment showed that in both cases the behavior functioned as a self-stimulatory response. Differential reinforcement, combined with a response interruption technique, produced large reductions in the hand biting of both girls and increases in the(More)
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