摘要
目的 总结下丘脑错构瘤的诊断和治疗。方法 40例下丘脑错构瘤 ,男∶女 =1 35∶1;发病年龄为 2个月至 5 1岁 ,中位数为 12 .5个月。 15岁以下儿童 32例 (80 % ) ,成人 (18岁以上 ) 4例 (10 % )。其临床主要特点为痴笑样癫痫和性早熟 ,少数还有其它类型癫痫、智力低下或合并先天畸形 ;其中单纯性早熟者 19例 (4 8% ) ,性早熟合并癫痫者 14例 (35 % ) ,单纯癫痫者 7例 (18% )。CT、MRI显示脚间池或垂体柄后上方有等信号 (等密度 )病变 ,注药无强化。手术治疗 2 7例 ,除 1例采用经纵板入路外 ,均采用经翼点入路切除错构瘤 ;γ刀治疗 8例 ;药物治疗 1例。结果 全切 14例(5 2 % ) ,其中 10例为单纯性早熟者 ,均治愈 ;大部切除 13例 ,症状明显好转 ,手术有效率为 97% ;单纯γ刀治疗的 5例中有效 1例 ,余 4例观察时间尚短 ,而难以判断 ;药物治疗性早熟有效 ,但对痴笑样癫痫及其它类型癫痫无效。结论 下丘脑错构瘤首选治疗为手术切除。
Objective To study the clinical feature and treatment of 40 cases of hypothalamic hamartomas.Methods 40 cases of hypothalamic hamartomas were reported, which included 23 males and 17 females. The onset age ranges from 2 month old to 51 years old. There are 32 cases(80%) children (younger than 15 years old)and 4 cases (10%) adults. The main clinical features of hypothalamic hamartoma are precocious puberty and gelastic seizures, some combine with other kind of seizures, mental retardation, or congenital abnormality. There are 19 cases (48%) present with simple precocious puberty, 14 cases (35%) with precocious puberty and epilepsy and 7 cases (18%) with simple epilepsy. MR scan revealed an isointense mass below the tuber cinereum extending into supersellar and interpeduncular cistern without enhancement. 27 cases were operated via a pterional approach except one case via a translaminaterminalis.8 cases were treated by gamma knife, one case were treated with GnRH analog.Results 14 cases hypothalamic hamartomas were totally removed (52%),10 of them who were presented with simple precocious puberty were cured. The other 13 cases that were removed gross-partially also improved symptom. The effective rate of operation was 97%; patients with simple precocious puberty can be cured if the hamartoma were total removed.Conclusions Microsurgery is the first choice of treatment for hypothalamic hamartomas.
出处
《中华神经外科杂志》
CSCD
北大核心
2002年第1期37-40,共4页
Chinese Journal of Neurosurgery
基金
北京市科委发展基金资助项目 ( 95 5 5 10 2 30 0 )
关键词
下丘脑
错构瘤
性早熟
痴笑样癫痫
外科手术
Hamartoma Hypothalamus Precocious puberty Gelastic seizure Microsurgery
