罗世祺 李春德 孙异临 100050 北京天坛医院神经外科(罗世祺、李春德)；北京市神经外科研究所(孙异临) 中华神经外科杂志 1998 0 14 3


    关键词：下丘脑；错构瘤；性早熟；痴笑样癫痫 期刊 zhsjwkzz 0 论 著 fur -->


    

摘要 目的：报告5例下丘脑错构瘤的临床表现、诊断及治疗。方法：分析5例下丘脑错构瘤的病史，临床表现及诊断、治疗情况，结合文献加以分析讨论。结果：5例皆在儿童早期发病，均有性早熟，其中3例有痴笑样癫痫，其它可有癫痫大发作，行为异常或智力减退，有2例手术治疗取得了较好疗效。病理可见神经元结构，电镜显示神经元内有分泌颗粒。结论：下丘脑错构瘤可以手术治疗，药物治疗对性早熟有效，而对痴笑样癫痫无效。

Hypothalamic hamartoma Luo Shiqi, Li Chunde, Sun Yilin. Departement of Neurosurgery. Beijing Tiantan Hospital, Beijing 100050

     Abstract Objective: To discuss the clinical features, diagnosis and treatment of hypothalamic hamartoma. Methods: The clinical features, diagnosis and treatment were discribed in 5 patients with reviewed of literatures. Results: Good results were achieved with surgical resection in 2 patients. Neuronal structure and neuro-secretory granules were found by pathology and electronic microscorpy respetively. Conclusion: Hypothalamic hamartoma can be treaed by surgical resection. Medical treatment are useful for precocious puberty, but not for gelastic seizure.

     Key words Hypothalamic hamartoma Precocious puberty Gelastic epilepsy

下丘脑错构瘤(hamartoma)又称下丘脑神经元错构瘤(hypothalamic neuronal hamartome)⁽¹⁾ 临床上极为罕见 ......

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