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KMID : 0386119970370030541
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Journal of the Korean Radiological Society
1997 Volume.37 No. 3 p.541 ~ p.545
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MR Imaging of Phenylketonuria
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Hong Hyun-Sook
Kim Dae-Ho
Lee Hae-Kyung
Kwon Kui-Hyang
Choi Deuk-Lin
Lee Dong-Hwan
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Abstract
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Purpose : The purpose of this study was to evaluate MR findings among patients with phenylketonuria(PKU).
Materials and Methods : Eleven patients with biochemically documented PKU underwent MR imaging ; In nine, the typical classic form was seen, and two were atypical. We evaluated signal intensity, the distribution of abnormal signal intensity, the extent of brain atrophy, and pissible clinical correlation between IQ scores of the patients and abnormal signal intensity.
Results : Varying degrees of symmetrical high signal intensity were noted on T2-weighted sequences in the parietal white matter of six patients ; the periventricular deep white matter was most seriously affected, but there was no evidence of brain atrophy. In on advanced case, high signal intensity of both the parietal and frontal lobes was seen on T2-weighted images, and brain atrophy and gyriform enhancement on contrast enhanced T1-weighted images. In five patients, finding were normal. No abnormality was found in the basal ganglia, brain stem or cerebellum. There was no correlation between IQ level and severity of high signal intensity.
Conclusion : Although MR findings were nonspecific, PKU patients showed symmetrical high signnal intensity, predominantly in the peritrigonal region. In the advanced case, the lesion, as seen on T2-weighted images, extended to the periventricular and subcortical white matter.
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KEYWORD
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Brain, MR, Brain, metabolism, Phenylketonuria,
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