Cognitive deficits in neurofibromatosis 1

J Child Neurol. 2002 Aug;17(8):605-12; discussion 627-9, 646-51. doi: 10.1177/088307380201700811.

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.

Publication types

  • Review

MeSH terms

  • Brain / pathology
  • Child
  • Child Behavior Disorders / diagnosis
  • Child Behavior Disorders / genetics
  • Humans
  • Intellectual Disability / diagnosis*
  • Intellectual Disability / genetics
  • Intelligence / genetics
  • Learning Disabilities / diagnosis*
  • Learning Disabilities / genetics
  • Neurofibromatosis 1 / diagnosis*
  • Neurofibromatosis 1 / genetics
  • Neuropsychological Tests
  • Quality of Life