脑神经元移行异常的MRI诊断

目的:报导38例脑神经元移行异常并总结本病MRI特点.材料和方法:38例小儿脑神经元移行异常患者.年龄3个月～14岁.主要临床表现为癫痫11例,智力减退17例,运动障碍16例.采用Toshiba 0.35T超导型MRI系统以自旋回波T1加权(T1WI)500/25ms及T2加权(T2WI)2000—4000/80ms,横断面及矢状面平扫,层厚5—10mm.结果:MRI特点:l)巨脑回/无脑回畸形20例.其中以无脑回为主者10例,表现额叶仅有几个扁平、粗大的脑回,顶枕叶脑表面光滑,无沟回显示.皮层增厚,白质变薄.以巨脑回为主者10例,主要表现脑回增宽,脑沟变浅.2)脑裂畸形16例.7例裂隙一端融合,9例裂隙分离.合并透明隔缺如者9例,合并多小脑回畸形者3例.3)灰质异位9例.孤立性2例,合并巨脑回/无脑回者3例,脑裂畸形者4例.结论:脑神经元移行异常MRI检查有典型征象,在本病诊断中起重要作用.

MRI in diagnosis of Neuron Migration Anomalies

Purpose: 38 cases of Neuron Migration Anomalies(NMA) diagnosed by MRI were reported.The purpose of this study is to manifest the characteristic MRI features. Materials andMethods: 38 children suffered from NMA, the age ranged from 3 months to 14 years. Themain symptoms included epilepsy(11 cases), mental retardation (17 cases), motor dysfunction(16 cases). A Toshiba O. 35 T superconductive MR system was used. Transaxial plane T1 andT2 weighted SE images were acquired with a head coil.Both sagittal and axial scannings wereperformed with slice thickness of 5 - 10mm. Results: Characteristics of MR Imaging: 1)Pachygyria/Agyria(20 cases) Agyria were apparent in 10 cases,absence of gyri and sulci inthe parietooccipital region combining with several bizarre and gross gyria in the frontal regionwere present with thickened cortex and thinnened white matter. Pachygyria were apparent inother 10 cases,in which broad gyria and shallow sulci were seen. 2) Schizencephaly(16 cases)Clefts with fused lips appeared in 7 cases; clefts with unfused lips in 9 cases. In each of 9cases, a small dimple was seen in lateral wall of ventricle where thd cleft communicated.Polymirogyria were noted within and adjacent to clefts. Absence of septum pellucidum in 9cases(56. 3% ). 3) Heterotopic gray matter(2 cases were solitary; 3 with pachygyria/ agyria; 4with schizencephaly). Conclution: MRI plays an important role in characterizing NMA moreexactly.