摘要
目的 分析苯丙酮尿症颅脑MRI异常表现。方法 用 0 5T超导磁共振机对临床检查和生化测定已确诊的 73例苯丙酮尿症患儿头颅进行扫描。结果 MRI发现异常者 71例。T2 WI上脑白质内散在斑片状高信号灶最为常见 (6 9例 ,94 5 % ) ;沿胼胝体向前后延伸并向双侧放射冠和半卵圆中心白质对称性扩展的弥漫性高信号病灶在T2 WI上也很常见 (5 1例 ,6 9 9% ) ;MRI还发现无胼胝体(3例 ,4 1% )、胼胝体发育不良 (17例 ,2 3 3% )、无透明隔 (2例 ,2 7% )、小脑下蚓部缺失 (1例 ,1 4% )等发育异常 ;有 5例 (6 8% )患儿颅腔小 ,尤以前额部明显 ,其中 2例 (2 7% )还出现脑皮质光滑 ,缺少脑沟回 ;弥漫性和局性灶脑萎缩 2 4例 (32 9% )。结论 苯丙酮尿症颅脑MRI表现为脑白质内斑片状和弥漫性高信号病灶、脑萎缩、脑发育异常和脑小畸形等。
Objective To analyze the brain abnormalities in phenylketonuria (PKU) patients by using MRI Methods Seventy three patients with clinically and biochemically documented phenylketonuria were studied with use of 0 5 T superconductive MRI Results Brain abnormalities were detected in 71 patients with PKU Patchy areas of increased signal intensity in white matter on T 2WI was the most common lesion (69 cases, 94 5%). The confluent high signal intensity extending symmetrically from corpus callosum to corona radiata was also often detected (51 cases, 69 9%) Absence of corpus callosum (3 cases, 4 1%), septum pellucidum (2 cases, 2 7%) , agenesis of corpus callosum(17 cases, 23 3%), and cerebellar vermis(1 case, 1 4%) were found MRI also demonstrated decreased cranial cavity predominantly in frontal region(5 cases, 6 8%), pachygyria(2 cases, 2 7%), and diffuse or local brain atrophy(24 cases, 32 9%) Conclusion Patchy and confluent areas of increased signal intensity in white matter, brain atrophy and developmental anomalies, and decreased cranial cavity are the abnormal appearances in brain of PKU
出处
《中华放射学杂志》
CAS
CSCD
北大核心
2000年第6期367-369,共3页
Chinese Journal of Radiology
基金
教育部留学回国人员工作资助!资金资助项目(1 991 40 8)
关键词
脑疾病
苯丙酮尿症
磁共振成像
Brain diseases
Phenylketonuria
Magnetic resonance imaging
