首都医科大学学报 ›› 2001, Vol. 22 ›› Issue (4): 327-329.

• 论著 • 上一篇    下一篇

痴笑、性早熟与先天性下丘脑错构瘤

孙异临1, 曲宝清1, 白勤1, 罗世琪2, 李春德2   

  1. 1. 北京市神经外科研究所;2. 首都医科大学附属北京天坛医院
  • 收稿日期:2000-09-13 修回日期:1900-01-01 出版日期:2001-10-15 发布日期:2001-10-15

Gelastic Precocious and Hypothalamic Hamartoma

Sun Yilin1, Qu Baoqing1, Bai qin1, Luo Shiqi2, Li Chunde2   

  1. 1. Beijing Neurosurgical Institute;2. Beijing Tiantan Hospital, Capital University of Medical Sciences
  • Received:2000-09-13 Revised:1900-01-01 Online:2001-10-15 Published:2001-10-15

摘要: 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14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.

关键词: 下丘脑错构瘤, 超微结构, 电镜

Abstract: To determine the relationship between the ultrapathology characteristic and clinical symptoms, signs of hypothalamic hamartoma, 14 cases of hypothalamic hamartoma were studied. Of them, there are 5 cases with precocious puberty; 4 cases with gelastic epilepsy; other cases with both precocious puberty and gelastic epilepsy. CT or MRI scan showed occupation mass in hypothalamic area. Total surgical resection were done in 14 cases. Biopsy specimen were examined with eptic and electron microscope. Ultrastructural research preved that the hypothalamic hamartoma is composed of neuroectoderm. The tumor parenchymal consisted of neurons and astrocytes. The tumor mesenchyme looked like neuropil. Electron microscopic examination showed that there were Golgi apparatus, microtubules, dense core vesicles and clear vesicles were found in numerous cytoplasm and cell processes. These morphologic features relate to precocious puberty and gelastic epilepsy. It is an important method to diagnosis hypothalamic neuronal hamartoma with eletron microscope technique.

Key words: hypothalamic hamartoma, ultrastructure pathology, electron microscope

中图分类号: