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用免疫细胞化学及超微结构形态学方法,探讨下丘脑错构瘤导致青春期前性早熟及痴笑样癫痫的发生机制。47例下丘脑错构瘤的手术标本进行促性腺激素释放激素特异性抗体(GnRH)的免疫组织化学和常规超薄切片电镜观察研究。免疫组化结果证实,伴有青春期前性早熟的错构瘤对GnRH抗体呈不同程度的阳性反应,说明其具有分泌促性腺激素释放因子的能力。电镜检查显示下丘脑错构瘤组织内神经细胞形态异常,神经毡内可见突触样结构内含大量清亮圆形的神经递质小泡和少量致密核心的分泌颗粒。研究结果表明,上述超微形态结构异常可能与痴笑性癫痫及青春期前性早熟相关。
Immunocytochemistry and ultrastructural morphology were used to investigate the mechanism of hypothalamic hamartoma leading to precocious puberty and craze-like epilepsy. Immunohistochemistry and conventional ultrathin section electron microscopy of 47 cases of hypothalamic hamartoma were performed immunohistochemistry and GnRH immunohistochemistry. Immunohistochemical results confirmed that hamartoma with precocious puberty had some degree of positive reaction to GnRH antibody, indicating that it has the ability to release gonadotropin-releasing factor. Electron microscopy showed abnormal neuronal morphology in the hypothalamus hamartoma. Synaptic structures within the neurofilament contained large numbers of clear and round neurotransmitter vesicles and a few dense core secreted granules. The results show that the above ultrastructural abnormalities may be associated with foolish epilepsy and precocious puberty.