Effect of Simvastatin on Cognitive Functioning in Children With Neurofibromatosis Type 1
Top Cited Papers
Open Access
- 16 July 2008
- journal article
- research article
- Published by American Medical Association (AMA) in JAMA
- Vol. 300 (3) , 287-294
- https://doi.org/10.1001/jama.300.3.287
Abstract
Neurofibromatosis type 1 (NF1) is a common autosomal-dominant genetic disorder (incidence 1:3000)1 caused by a mutation in the gene encoding neurofibromin, a protein that activates the hydrolysis of RAS-bound guanosine triphosphate.2 Neurofibromatosis type 1 is characterized by various neurocutaneous manifestations, problems in fine and gross motor functioning,3 as well as the frequent occurrence of cognitive disabilities. Children with NF1 have a lower mean IQ (86-94) with particular deficits in visual-spatial skills, nonverbal long-term memory, executive functions, and attention.4-7 These problems have a large impact on school performance of children with NF1.4 It has been suggested that the cognitive and motor deficits in children with NF1 are related to hyperintensities on T2-weighed magnetic resonance imaging of the brain3,8 that are characterized by high apparent diffusion coefficients (ADC values),9 but some studies failed to confirm this relationship.10Keywords
This publication has 22 references indexed in Scilit:
- Functional MRI of visual–spatial processing in neurofibromatosis, type INeuropsychologia, 2004
- Behavioural, academic and neuropsychological profile of normally gifted Neurofibromatosis type 1 childrenJournal of Intellectual Disability Research, 2004
- Effects of statins and farnesyltransferase inhibitors on the development and progression of cancerCancer Treatment Reviews, 2004
- Neurofibromatosis type 1Neurology, 2003
- Cerebellar Lesions and Prism Adaptation in Macaque MonkeysJournal of Neurophysiology, 1999
- Neurofibromatosis type 1 in childhood: correlation of MRI findings with intelligence.Journal of Neurology, Neurosurgery & Psychiatry, 1995
- Specific learning disability in children with neurofibromatosis type 1Neurology, 1994
- Isoprenoid addition to Ras protein is the critical modification for its membrane association and transforming activity.Proceedings of the National Academy of Sciences, 1992
- Identification of the neurofibromatosis type 1 gene product.Proceedings of the National Academy of Sciences, 1991
- National Institutes of Health Consensus Development Conference Statement: neurofibromatosis. Bethesda, Md., USA, July 13-15, 1987.1988