带状型灰质异位合并分离型脑裂畸形和透明隔部分缺如的临床和影像学特点(附1例报告)  

Clinical and imaging features of subcortical band heterotopia complicated with open-lip schizencephaly and septum pellucidum partial deletion(report of 1 case)

作　　者：杨中原[1] 任传成[1] 

机构地区：[1]复旦大学附属上海市第五人民医院神经内科,200240

出　　处：《临床神经病学杂志》2013年第2期144-146,共3页Journal of Clinical Neurology

摘　　要：目的探讨带状型灰质异位(SBH)合并分离型脑裂畸形(OS)和透明隔部分缺如(SPPD)的临床和影像学特点。方法回顾性分析1例SBH合并OS和SPPD患者的临床资料。结果本例患者幼年起病,以右侧肢体发育短小、右侧肢体痉挛性高肌张力、右侧肢体运动功能障碍,以及青年期发病的癫痫发作、发作性意识障碍为主要症状。头颅CT平扫示左侧顶叶脑裂畸形伴灰质异位。头颅MRI平扫示左侧顶叶脑裂畸形,与左侧脑室相通,裂隙周边为连续迂曲状T1WI低信号、T2WI高信号影(与灰质信号一致),左侧脑室扩大,部分透明隔缺如。结论 SBH合并OS和SPPD患者常见表现为癫痫、精神智能发育迟滞和神经系统功能缺失等,其影像学特点为侧脑室旁与灰质之间的白质中出现边界清晰、规则的灰质,呈"双皮质"样;同时可见OS和SPPD。Objective To explore the clinical and imaging features of subcortical band heterotopia （SBH） complicated with open-lip schizencephaly （OS） and septum pellucidum partial deletion （SPPD）. Methods The clinical data of one SBH patient complicated with OS and SPPD was analyzed retrospectively. Results The patient with juvenile onset, the tight limb development in short, the spastic muscle tone of the right side, the motor dysfunction of the right side, and adolescent onset seizures, episodic disturbance of consciousness as the main symptoms. Cranial CT scan showed that left parietal schizencephaly with gray matter heterotopia. Cranial MRI scan showed that left parietal schizencephaly connected with the left ventricle; the schizencephaly was surrounded by a continuous tortuous-like low signal in TIWI and high signal in T2WI intensity （consistent with gray matter signal ） ; the left ventricle expanded; part of the septum pellucidum dysplasia. Conclusions The manifestations of SBH patient complicated with OS and SPPD are epilepsy, mental retardation, growth retardation and nervous system dysfunction ,etc. The imaging features are clear and regular gray matter appeared in the white matter between the lateral ventricles and gray matter, just like ＂double cortex＂, besides OS and SPPD.

关 键 词：灰质异位 脑裂畸形 临床特点 MRI 

分 类 号：R742.89[医药卫生—神经病学与精神病学]