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为探讨人下丘脑错构瘤的超微病理特点及其与临床症状、体征的关系 ,将 1 4例下丘脑错构瘤患者的手术切除新鲜活检组织 ,在光学显微镜及电子显微镜下检查。结果 :光镜下可见散在大小不等的神经细胞 ,部分病例胶质细胞增生。电镜检查可见神经细胞形态不一 ,胞核相对较大 ,胞质内细胞器丰富 ,可见少量致密核心颗粒 ;间质内见神经毡结构 ,细胞突起终末有突触形成并有大量兴奋性神经递质小泡。超微结构证实下丘脑错构瘤由神经上皮成分构成 ,可见发育不成熟的神经元并有胶质细胞增生。细胞突起内见神经递质小泡及致密核心的神经分泌颗粒 ,这些形态特点与患儿的性早熟及痴笑样癫痫有关。结果提示电镜是诊断下丘脑错构瘤的重要手段
In order to investigate the ultrastructural features of human hypothalamic hamartoma and its relationship with clinical symptoms and signs, surgical resection of fresh biopsies in 14 patients with hypothalamic hamartoma was performed under light microscope and electron microscope. Results: Light microscope showed scattered nerve cells in different sizes, in some cases glial cell proliferation. Electron microscopy showed nerve cells in different shapes with relatively large nuclei and abundant organelles in the cytoplasm. A small amount of dense core particles were observed. Neurofilament structure was found in the interstitium, and synapses formed at the terminal of the cell process with a large number of excitatory neurotransmitters Small bubbles. Ultrastructure confirmed hypothalamic hamartoma neuroepithelial composition, we can see immature neurons and glial cell proliferation. Neurons within the neurites, neurotransmitter vesicles and dense core of the neurosecretory particles, these morphological characteristics and children with precocious puberty and craze-like epilepsy. The results suggest that electron microscopy is an important means of diagnosis of hypothalamic hamartoma