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MR imaging revealed a high frequency of supra-and infratentorial abnormalities.MR imaging grade,which was based on areas of high signal intensity on T2-weighted images,showed statistically significant correlation with long-term biochemical control and neuropsychologic test results but not with intelligence quotient or VEP's.H-1 MR spectroscopy revealed normal metabolite levels,except for increase PHE levels.It also showed enlarged cerebrospinal fluid-like compartments in affected white matter,related to plasma and brain concentrations of PHE and MR imaging grades.A synergistic use of MR imaging and MR spectroscopy may help elucidate both the pathogenesis of brain dysfunction and clinical treatment policies in PKU. |
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