冷慧，付静，梁乐，刘瑛

（北京市海淀医院，北京大学第三医院海淀院区，北京100080）

灰质异位的临床病理学特征研究

冷慧，付静，梁乐，刘瑛

（北京市海淀医院，北京大学第三医院海淀院区，北京100080）

目的探讨灰质异位病理学诊断的客观依据。方法分析17例灰质异位手术切除标本病理及临床资料，辅以免疫组化方法，计数灰质异位灶中神经元的数量和类型。结果本组病例中灰质异位术前MRI确诊为3例，其余14例（82.4%，14/17）均依靠镜下诊断。大体改变有三型：（1）白质中孤立的皮质样结节；（2）位于皮质下、呈结节状或舌状与皮质相连；（3）大脑皮层增厚，皮白质分界不清。镜下部分灰质异位结节边界清，结节中神经元排列紊乱，无极向，无正常皮质分层结构；神经元可表现退变、坏死、形态不成熟；免疫组化GFAP可显示部分灰质异位结节的轮廓，神经元核抗原（neuronal nuclei，NeuN）、微管相关蛋白2，（microtubule associated protein-2，MAP-2）染色显示神经元数量减少且其中不成熟神经元比例增高，差异有显著统计学意义（t=-3.66，P＜0.01）。结论灰质异位病变范围广泛时可于影像学检测中发现，但多数的灰质异位诊断仍需依靠病理组织学确诊，免疫组化GFAP有利于观察灰质异位灶形态，NeuN和Map-2强阳性细胞计数能为灰质异位诊断提供客观依据。

灰质异位；病理学；免疫组化

〔Abstract〕Objective To explore the objective basis for the pathological diagnosis of gray matter heterotopia.M ethods The pathological and clinical data of 17 cases of gray matter heterotopia resection were analyzed，and supplemented by immunohistochemical method，the number and types of neurons in the gray matter heterotopia were counted.Results Only 3 cases of gray matter heterotopias were diagnosed by MRI before surgery in this group.The remaining 14 cases（82.4%，14/17）were all diagnosed by microscopic diagnosis.There are three types of gross performance：（1）isolated gray matter nodule in white matter.（2）Gray matter heterotopia is located in the subcortical and show nodular or tongue connected to the cortex.（3）Cerebral cortical is thickening.The boundary is not clear between gray matter and white matter.Microscopically，the boundary of some heterotopic gray matter is clear，neuronal arrangement disorder within the heterotopic gray matter，polarity of dendrite and axon are disorder.There isn't normal cortical layer structure.Neurons show degenerative changes，necrosis and immature forms.Immunohistochemistry GFAP can show the outline of some gray matter heterotopic nodules.NeuN and Map-2 staining showed a decrease in the number of neurons and a increase in proportion of immature neurons.The difference was statistically significant（t=-3.66，P＜0.01）.Conclusion A wide range of gray matter lesions can be found in imaging detection，But most of the heterotopia diagnosis still depends on pathology.GFAP is valuable for the observation of the shape of the graymatter heterotopias.Counting NeuN and Map-2 positive cells can provide basis for the diagnosis of gray matter heterotopia.

〔Keywords〕gray matter heterotopia；pathology；immunohistochemistry

灰质异位（gray matter heterotopia，GMH）是神经元迁移异常所导致的大脑发育畸形，临床可导致频繁发作的难治性癫痫。随着影像学和手术治疗癫痫技术的进展，GMH受到越来越多的关注，成为难治性癫痫研究的热点之一。但已有的相关研究大多是关于GMH影像学改变、脑电图特征及相关的基因异常等方面的，而病理组织形态学方面的研究较少。……