HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH TABLE OF CONTENTS		QUICK SEARCH:   [advanced] Author: Keyword(s): Year:  Vol:  Page:

This Article Figures Only Full Text Full Text (PDF) Correspondence: Submit a response Alert me when this article is cited Alert me when Correspondence are posted Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Hyman, S.L. Articles by North, K.N. Search for Related Content PubMed PubMed Citation Articles by Hyman, S.L. Articles by North, K.N. Related Collections All Imaging MRI All Neuropsychology/Behavior All Pediatric Developmental disorders Mental retardation Neurofibromatosis

Natural history of cognitive deficits and their relationship to MRI T2-hyperintensities in NF1

From the Neurogenetics Research Unit (S.L. Hyman and Dr. North), TY Nelson Department of Neurology (D.S. Gill), Developmental Cognitive Neuropsychology Research Unit (P. Joy), and Department of Radiology (A. Steinberg and S.V. Gibikote), The Children’s Hospital at Westmead (Royal Alexandra Hospital for Children), Sydney; Department of Pediatrics and Child Health, Faculty of Medicine (S.L. Hyman and Dr. North), University of Sydney; and Department of Psychology (Dr. Shores), Macquarie University, Sydney, Australia.

Address correspondence and reprint requests to Professor Kathryn North, Neurogenetics Research Unit, Children’s Hospital at Westmead, Locked Bag 4001, Westmead NSW 2145, Sydney, Australia; e-mail: kathryn{at}chw.edu.au

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.

Objective: To determine the natural history of cognitive functioning and MRI T2-hyperintensities from childhood into adulthood, and whether changes in MRI T2-hyperintensities over time are predictive of changes in cognitive functioning.

Methods: The authors conducted a prospective longitudinal study of a cohort of 32 patients with NF1 and 11 unaffected sibling controls. All patients underwent neuropsychological assessments and 27 children underwent MRI examinations. The patients were then reassessed after an 8-year period.

Results and Conclusions: There was no improvement in cognitive ability as the children with NF1 developed into adulthood compared with controls. Despite significant decreases in the number, size, and intensity of the T2-hyperintensities over the 8-year period, these changes were not associated with changes in cognitive ability. T2-hyperintensities in the cortex or subcortical or deep white matter are more frequent with age and these lesions are likely to have a different pathology than basal ganglia lesions. The best predictor of cognitive dysfunction in adulthood was the presence of T2-hyperintensities in childhood, rather than current lesion status. There is a limited time window (<18 years) in which the presence of T2-hyperintensities can be used as biologic markers of cognitive dysfunction.

This article has been cited by other articles:

				S. L Hyman, D. S Gill, E. A. Shores, A. Steinberg, and K. N North T2 hyperintensities in children with neurofibromatosis type 1 and their relationship to cognitive functioning J. Neurol. Neurosurg. Psychiatry, October 1, 2007; 78(10): 1088 - 1091. [Abstract] [Full Text] [PDF]

				S.L. Zamboni, T. Loenneker, E. Boltshauser, E. Martin, and K.A. Il'yasov Contribution of Diffusion Tensor MR Imaging in Detecting Cerebral Microstructural Changes in Adults with Neurofibromatosis Type 1 AJNR Am. J. Neuroradiol., April 1, 2007; 28(4): 773 - 776. [Abstract] [Full Text] [PDF]

				S. L. Hyman, A. Shores, and K. N. North The nature and frequency of cognitive deficits in children with neurofibromatosis type 1 Neurology, October 11, 2005; 65(7): 1037 - 1044. [Abstract] [Full Text] [PDF]

				R. Feldmann, J. Denecke, M. Grenzebach, G. Schuierer, and J. Weglage Neurofibromatosis type 1: Motor and cognitive function and T2-weighted MRI hyperintensities Neurology, December 23, 2003; 61(12): 1725 - 1728. [Abstract] [Full Text] [PDF]