Relationship between T2-weighted hyperintensities (unidentified bright objects) and lower IQs in children with neurofibromatosis-1

✍ Scribed by Denckla, M. B.; Hofman, K.; Mazzocco, M. M. M.; Melhem, E.; Reiss, A. L.; Bryan, R. N.; Harris, E. L.; Lee, J.; Cox, C. S.; Schuerholz, L. J.

• Publisher: John Wiley and Sons
• Year: 1996
• Tongue: English
• Weight: 25 KB
• Volume: 67
• Category: Article
• ISSN: 0148-7299
• DOI: 10.1002/(sici)1096-8628(19960216)67:1<98::aid-ajmg17>3.0.co;2-k

✦ Synopsis

To address the controversy regarding the rela tionship bet ween cognitive impairment (lowering of I&) and magnetic resonance imaging (MRI) characteristics (T2-weighted hyperintensities or unidentified bright objects [UBOs]) in children with neurofibromatosis-1 (NF-l), we used a pairwise NF'-1/ sibling design; we set out to predict the lowering of I& in each child with NF-1 as a discrepancy from the I& of an unaffected sibling (D-SIQ). Our multiple regression model included the age of the child with NF-1, familial or sporadic nature of the NF-1, number of locations in the child's brain occupied by T2-weighted hyperintensities (UBOs), and the volumetric percentage of brain tissue occupied by T2-weighted hyperintensities (UBOs). Only the number of locations occupied by UBOs accounted for I& lowering (D-SIQ) in children with "-1 (42% of the variance in D-SIQ). This is the first report to confirm that a continuum of lowered IQs in NF-1-affected children exists in relation to the distribution of UBOs (range &7), not just presence (vs. absence) of any UBOs.