RT Journal Article
SR Electronic
T1 Diffusion Tensor Images in Children with Early-Treated, Chronic, Malignant Phenylketonuric: Correlation with Intelligence Assessment
JF American Journal of Neuroradiology
JO Am. J. Neuroradiol.
FD American Society of Neuroradiology
SP 1569
OP 1574
VO 25
IS 9
A1 Steven Shinn-Forng Peng
A1 Wen-Yih Isaac Tseng
A1 Yin-Hsiu Chien
A1 Wuh-Liang Hwu
A1 Hon-Man Liu
YR 2004
UL http://www.ajnr.org/content/25/9/1569.abstract
AB BACKGROUND AND PURPOSE: Diffusion tensor (DT) images can provide information about the nature of white matter changes, including axonal loss and demyelination. We applied DT imaging to verify white matter changes in patients with malignant phenylketonuria (PKU) and to correlate the findings with clinical intelligence quotients (IQs).METHODS: We compared DT images with T2-weighted images in 12 patients with early-treated, chronic, stable malignant PKU and 12 age-matched control subjects. DT parameters included first, second, and third eigenvalues (EV1–3), apparent diffusion coefficients (ADCs), and fractional anisotropy (FA). Regions of interest were placed the frontoparietal, parieto-occipital, frontal and central white matter and in the anterior and posterior corpus callosum. Eight patients older than 3 years underwent IQ assessment including verbal, performance, and full-scale IQ tests.RESULTS: In the eight patients older than 3 years, no definite abnormal signal intensity changes were found on T2-weighted images. EV2, EV3, and FA of the parieto-occipital white matter were significantly different in patients and control subjects older than 3 years. EV3 and ADC of the parieto-occipital white matter were significantly and negatively correlated with verbal IQ (r = −0.79, P = .04) and performance IQ (r = −0.93, P = .03). FA of the parieto-occipital central white matter was positively correlated with verbal IQ (r = 0.75, P = .05).CONCLUSION: Though treated early, patients with chronic, stable malignant PKU had abnormal DT findings in the parieto-occipital central white matter. EV2, EV3, and FA maps are potential tools for demonstrating brain changes due to malignant PKU.