脑灰质异位症的临床及影像学诊断  

Clinical and imaging diagnosis of gray matter heterotopia

作　　者：张玉珍[1] 尹秋凤[1] 蔡静[1] 郑慧[1] 刘明[1] 汪登斌[1] ZHANG Yuzhen;YIN Qiufeng;CAI Jing;ZHENG Hui;LIU Ming;WANG Dengbin(Department of Radiology,Xinhua Hospital Affiliated to Shanghai Jiaotong University,School of Medicine,Shanghai 200092,P.R.China)

机构地区：[1]上海交通大学医学院附属新华医院放射科,上海200092

出　　处：《医学影像学杂志》2021年第9期1449-1453,共5页Journal of Medical Imaging

基　　金：上海市科研技术委员会科研计划项目(编号:18411967500);国家重点研发计划(子课题)(编号:2017YFC0109003)。

摘　　要：目的探讨脑灰质异位症(GMH)的临床表现及影像诊断价值。方法选取并分析83例GMH临床及影像学表现。结果临床表现为癫痫发作46例,智力落后伴癫痫12例,头晕头痛4例,胎儿期发现异常6例。CT平扫5例,MRI检查59例(增强12例),CT及MRI均检查19例(增强7例)。室管膜下、白质内、带状及混合性GMH病例数分别为60、4、3及16例。表现为相应部位单发或多发,异常结节样、团块状或带状灰质密度或信号。主要合并症为侧脑室扩大、大枕大池、蛛网膜囊肿、脑裂畸形、皮层发育不良、脑软化及腔隙灶,胼胝体发育不良、静脉畸形及颅缝早闭等。合并症≥2项者33例,不伴合并症16例。结论GMH主要临床表现以全身阵挛强直癫痫为主。MRI平扫T 1WI、T 2WI及DWI序列,即可进行GMH范围及分布的明确诊断,3D薄层T 1WI及重建可为诊断及手术治疗者提供更全面影像信息。Objective To summarize the clinical presentation and image characteristics of gray matter heterotopia(GMH).Methods Clinical presentation;imaging features of computed tomography(CT)and magnetic resonance imaging(MRI)of 83 GMH cases were retrospectively reviewed.Results Patients presented with epilepsy(n=46),mental retardation and epilepsy(n=12),headache and dizziness(n=4),follow-up study after abnormal findings in prenatal ultrasound(n=6),etc.CT,MRI and both examinations were performed in 5,59(12 with contrast enhancement)and 19 cases(7 with contrast enhancement)respectively.60 cases were periventricular nodular heterotopias(PNH).4 cases were subcortical GMH(SGMH).3 cases were subcortical band heterotopias(SBH).16 cases were mixed GMH.The imaging findings were single or multiple,abnormal nodular or banded GM density or signal intensity in the corresponding position.The associated abnormalities were lateral ventricles enlargement,mega cisterna magna and subarachnoid cyst,schizencephaly,cortical dysplasia,encephalomalacia and lacunar infarct,corpus callosum dysplasia,venous malformation and craniosynostosis.There were 33 cases with over 2 associated abnormalities,while 16 cases without associated abnormalities.Conclusion GMH is a rare congenital malformation of cortical development with Tonic-clonic seizures.Conventional MRI sequences,T 1WI,T 2WI and DWI should be suggested in localizing GMH,3D thin section T 1WI sequence and reformation can provide much imaging information in diagnosis and clinical treatment.

关 键 词：灰质异位症 癫痫 磁共振成像 

分 类 号：R742.8[医药卫生—神经病学与精神病学] R445[医药卫生—临床医学]