Neurofibromatosis type 1 (NF1) is a neurocutaneous disorder with a prevalence of approximately 1 in 3500 (Huson & Hughes, 1994). Patients with NF1 have a wide variety of manifestations and a broad range of severity. One of the most common complications of NF1 in childhood is cognitive/neuropsychological dysfunction. These deficits are wide ranging and can be responsible for significant difficulties in daily activities, especially in the academic environment.
Knowledge regarding the cognitive profile of children with NF1 has increased dramatically over the past decade. Initially mental retardation was thought to be highly prevalent in NF1 patients, however large population studies have shown that there is only a slight increase in the incidence of mental retardation (North, 1999). Many studies have shown a slight reduction of general intellectual functioning, with IQ falling within the average to low average range (Ozonoff, 1999). Despite only minimal influence of the NF1 gene upon general intellectual functioning, more significant specific cognitive deficits have been reported in several areas, including perception, attention, executive functioning and language skills. These neuropsychological deficits result in a high prevalence of academic difficulties, and have important implications for schooling, career choice, and overall quality of life.
