Affiliation:
1. Neurogenetics Research Unit Children's Hospital at Westmead, Department of Paediatrics and Child Health, University of Sydney, Sydney, Australia
Abstract
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 the patient group is within 1 SD of the population mean. Academic difficulties are common, as are specific deficits in visuospatial ability, executive function, expressive and receptive language, and attentional skills. Behavioral and psychosocial problems have a major impact on quality of life, although there are few objective studies in this area. Current research is focusing on the pathogenesis of the disorder. Clinical studies have identified possible radiologic and pathologic markers for cognitive deficits in neurofibromatosis 1, which can now be explored in animal models. (J Child Neurol 2002;17:605-612).
Subject
Neurology (clinical),Pediatrics, Perinatology and Child Health
Reference55 articles.
1. Review Article : Neurofibromin in the Brain
2. Review Article : Molecular and Cellular Mechanisms Underlying the Cognitive Deficits Associated With Neurofibromatosis 1
3. Cognitive function and academic performance in neurofibrornatosis 1: Consensus statement from the NF1 Cognitive Disorders Task Force
4. North KN: Cognitive function and academic performance, in Friedman JM, Gutmann DH, MacCollin M, Riccardi VM (eds): Neurofibromatosis: Phenotype, Natural History, and Pathogenesis, 3rd ed. Baltimore , Johns Hopkins University Press, 1999, 162-189.
5. Cognitive impairment in neurofibromatosis type 1
Cited by
97 articles.
订阅此论文施引文献
订阅此论文施引文献，注册后可以免费订阅5篇论文的施引文献，订阅后可以查看论文全部施引文献