目的探讨儿童下丘脑错构瘤的 临床及MRI表现特征，以提高对本病的认 识。方法 回顾性分析18例经手术病理证 实的儿童下丘脑错构瘤的临床特点和MRI 表现，其中男10例，女8例，年龄6个月 至12岁。14例进行了MRI平扫和增强，4 例仅做了MRI平扫。结果 主要临床表现 为痴笑样癫痫、性早熟。首发症状为痴 笑样癫痫9例，性早熟5例，肢体抽搐2 例，运动发育迟缓2例。MRI表现为鞍上 下丘脑附近区域单发结节，附着于灰结 节和乳头体，呈类圆形或椭圆形，直径 ＜15mm者14例，＞15mm者4例，最大直径 24mm。病灶同脑灰质比较，T1WI呈等或 稍低信号，T2WI呈等或稍高信号，信号 均匀。18例中2例合并坏死囊变。14例增 强扫描肿块均未见强化。结论 儿童下丘 脑错构瘤的临床及MRI表现具有特征性， 当患儿出现痴笑样癫痫或性早熟，MRI表 现为下丘脑附近区域类圆形结节影且无 强化，应首先考虑本病。

Objective To study the clinical manifestations and MRI characteristics of hypothalamic hamartoma. Methods The clinical manifestations and MRI findings of 18 chlidren(included 10 boys and 8 girls,ranging in age of onset from 6 months to 12 years) with hypothalamic hamartomas proved by surgery and pathology were retrospectively reviewed. 14 patients were examined with precontrast and postcontrast MR imaging,only 4 were scanned without contrast study. Results The main clinical features of the hypothalamic hamartomas were due to gelastic epilepsy and precocious puberty. Gelastic epilepsy was found as initial clinical symptom in 9 cases, precocious puberty in 5 cases, limbs spasm in 2 cases,motor developmental retardation in 2 cases. MRI showed abnormal single nodules located in the suprasella and hypothalamic regions with round or elliptical shape. All mass had sessile attached to the part of tuber cinereum and mammillary body. The dimeters of the mass ＜15mm and ＞15mm in 14 and 4 cases. On MRI, the mass was isointense or slightly hypointense on T1WI and isointense or slightly hyperintense or hyperintense on T2WI relative to gray matter,16 of 18 cases the singnal was very homogeneous, two of them combined with necrosis. And no enhancement was observed. Conclusion There were characteristic findings in the clinical manifestations and MRI to evaluate hypothalamic hamartomas. Hypothalamic hamartomas should be considered firstly when patient have the symptom of gelastic epilepsy and precocious puberty,as well as non-enhanced round shape nodules located in the suprasella and hypothalamic regions