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目的使用磁共振扩散加权成像(DWI)技术研究苯丙酮尿症(phenylketonuria,PKU)患者脑白质内病灶的扩散特点,并观察表观扩散系数(apparent diffusion coefficient,ADC)的变化。资料与方法对8例临床确诊的PKU患儿及性别、年龄相匹配的8名健康儿童(对照组)行T1WI、T2WI及DWI,测量侧脑室后角T2WI异常信号区及放射冠正常白质的ADC值和对照组相应部位的ADC值。结果在T2WI上,所有未经治疗的PKU患儿均表现为非占位性、斑片状、条带状高信号,在X、Y、Z3个方向同时施加扩散梯度的跟踪ADC图(trace APC map)上显示更清晰。患儿大脑白质病灶X、Y、Z3个方向的ADC值明显低于对照组相应部位脑白质的ADC值,两者差异存在统计学意义(P<0.05)。平均ADC值比对照组相应部位减少23%,侧脑室后角扩散各向异性消失(F=0.195,P=0.825)。结论未经治疗的PKU白质异常在DWIav上显示更清晰,DWI可提供PKU患儿脑白质病灶的病理信息。
Objective To investigate the characteristics of diffusion in focal leukoplakia of patients with phenylketonuria (PKU) using diffusion weighted imaging (DWI) and observe the changes of apparent diffusion coefficient (ADC). Materials and Methods 8 cases of clinically diagnosed children with PKU and 8 healthy children (control group) of gender and age matched for T1WI, T2WI and DWI were measured. The ADC of T2WI in the posterior horn and the normal white matter ADC value of the corresponding part of the control group. Results All patients with untreated PKU showed non-occupying, patchy, and stripe-like signals on the T2WI. The trace ADCs of diffusion gradients were simultaneously applied in X, Y and Z directions map) clearer. The ADC values of X, Y, and Z directions in white matter lesions of children with cerebrovascular disease were significantly lower than those of the control group. The differences between the two groups were statistically significant (P <0.05). The average ADC value decreased by 23% compared with the corresponding part of the control group, and the anisotropy of lateral ventricular posterior diffusion disappeared (F = 0.195, P = 0.825). CONCLUSIONS: Untreated PKU white matter abnormalities appear to be clearer on DWIav, and DWI provides pathological information on white matter lesions in children with PKU.