下丘脑错构瘤214例临床特征分析

Clinical characteristics of 214 cases on hypothalamic hamartoma

目的分析下丘脑错构瘤的临床特征。方法回顾分析1994年1月至2008年5月北京天坛医院诊治的214例下丘脑错构瘤的临床资料。结果性别：男性多于女性，男女之比为1．52：1；有性早熟的115例中，男女之比为1．09：1；有癫痫的123例中男女之比为2．15：1；有痴笑样癫痫的96例中男女之比为2：1；同时表现为性早熟及癫痫的38例中男女之比为1．38：1。发病年龄：200例有症状的病例，平均发病年龄为34．5个月；发病年龄43岁者157例，占78．5％；单纯性早熟者77例（38．5％），平均发病年龄16．5个月，〈3岁者67例，占87％；表现有性早熟者115例（57．5％），平均发病年龄为17．63个月，发病〈3岁者99例，占86．1％；有痴笑样癫痫者96例（48％），平均为26．14个月，〈3岁者有78例，占81．3％；所有癫痫者[痴笑样癫痫和（或）癫痫大、小发作]123例（61．5％），平均发病年龄为3．81岁，≤3岁前发病者有90例，占73．2％。结论下丘脑错构瘤多数在婴幼儿期发病，男性多于女性。

Objective To study the clinical characteristics in hypothalamic hamartoma. Methods A retrospective review of 214 patients with hypothalamic hamartoma was performed between January 1994 and May 2008. Results There were more patients in male and the ratio of male and female was 1.52： 1. The rate of male and female in 115 patients with precocious puberty was 1.09： 1, in 96 patients with gelastic seizures was 2： 1, in 123 patients with epilepsy was 2. 15： 1, in 38 patients with precocious puberty and epilepsy was 1.38： 1. There were 14 asymptomatic patients, the median age at the time of symptomatic was 34. 5 months. In the rest 200 patients with symptomatic, there were 157 patients younger than 3 year old （78.5%） ; the median onset age in 77 patients with simplicity precocious puberty was 16. 5 months, in 115 patients with precocious puberty was 17.63 months, in 96 patients with gelastic seizures was 26. 14 months, in 123 patients with all type epilepsy was 3. 18 yr. Conclusions The onset age of hypothalamic hamartoma is in younger children and more found in male.