17例灰质异位的临床及病理观察

Clinical and pathological observation of7 cases with gray matter heterotopia

关键词:  灰质异位  病理学  癫痫  免疫组织化学

Key words:  gray matter heterotopia  pathology  epilepsy  immunohistochemistry

摘要:

目的　探讨灰质异位的临床症状，治疗方法、病理学特点及意义。方法　回顾性总结北京市海淀医院2008年6月至2015年6月共17例灰质异位的临床资料，所有病例临床表现均为难治性癫痫，脑电图检测到痫样放电，MRI检测出白质内异常信号，确诊灰质异位3例。17例均行手术治疗，观察手术切除脑组织样本的大体及镜下的病理特点，并对预后进行评估。结果7例病人中，灰质异位多发生于单侧半球（13例），局限于颞叶9例，弥漫多发14例。灰质异位灶镜下组织学结构分为两种类型，异位神经元形态为梭形神经元、中小型神经元或未成熟神经元，异位灶内少见有胶质细胞。免疫组化染色显示异位神经元NeuN、MAP-2阳性，GFAP清晰标记出结节轮廓。所有病人均伴有难治性癫痫，且致痫皮质区域呈FCDⅠ级，所有病人均采用手术切除病灶加致痫灶，随访18～36个月，1例为门诊随访，采用Engel's评级评估显示，Ⅰ级13例，Ⅱ级3例，Ⅲ级1例。结论　灰质异位为大脑发育畸形中的一种，可致患者难治性癫痫及智力减退，MRI检测可辅助诊断，但最终确诊仍需要依靠病理组织学与免疫组化染色相结合，手术切除灰质异位及周围致痫灶，可有效控制癫痫发作，术后效果较好。

Abstract:

Objective To investigate the clinical symptoms，therapeutic methods，and pathological characteristics and significance of the gray matter heterotopia.Methods Clinical data of7 cases of heterotopic gray matter in Beijing Haidian Hospital from June008 to June015 were retrospectively reviewed.In all cases，the clinical manifestations were intractable epilepsy and epileptic discharges were detected by EEG.MRI detected abnormal signals in white matter and cases of heterotopic gray matter were diagnosed.Surgical treatment was performed in7 cases.We observed the gross and microscopic features of the brain tissue samples and evaluated the effect after surgery.Results In7 cases，there were3 cases that heterotopic gray matter occurred in the unilateral hemisphere，confined to the temporal lobe in 9 cases and diffuse multiple (14 cases).EEG detection was not specific in the diagnosis.The high signals in white matter can be detected by MRI，and only cases were directly diagnosed.The rest cases were diagnosed by pathological diagnosis.There were two types of tissue structure in the gray matter ectopic foci.The morphology of ectopic neurons was spindle shaped neurons，small and medium sized neurons or immature neurons.Immunohistochemical staining showed that MAP-2 and NeuN were positive in ectopic neurons，and the nodule contour was clearly marked by GFAP.All patients were associated with refractory epilepsy，and the area of the epileptic cortex was FCDⅠ.All patients were followed up for8-36 months，1 patient was outpatient follow-up，and patients were reviewed for Engel grade I (13 cases)，grade II (3 cases)，grade III (1 case).Conclusion Gray matter heterotopia is a kind of brain developmental malformation.It can lead to patients with intractable epilepsy and mental decline.But the final diagnosis still depends on histopathology and immunohistochemical staining.Surgical excision of heterotopic gray matter and peripheral epileptic foci can effectively control the seizure.The effect will be better after the operation.