目的 分析下丘脑错构瘤的临床特征及核磁共振成像表现，提高对下丘脑错构瘤的诊断水平。方法 回顾性分析本院 收治的经手术及病理证实的8例下丘脑错构瘤的临床资料。其中男5例，女3例，发病年龄最大为14岁，最小为1岁。 8例均行MRI平扫及增强扫描。结果 8例病例中，临床表现为性早熟者5例，痴笑癫痫者3例，其他类型癫痫者4例， 多饮多尿1例。MRI表现为鞍上下丘脑区圆形或不规则形的结节影，与灰结节或乳头体相连，直径为12mm-25mm， T1WI上与脑灰质信号一致，T2WI上呈等信号或稍高信号，信号均匀，增强后未见强化。结论 下丘脑错构瘤的临床 表现及影像学表现具有特征性，MRI表现结合临床特征能做出该病较为明确的诊断。

Objective To improve the diagnosis level of MRI for hypothalamic hamartomas by analyzing its clinical and MRI features. Methods MRI and clinical data of 8 patients (included 5 boys and 3 girls, age from 1 to 14 years old.) with hypothalamic hamartomas proved by operation and pathology were reviewed retrospectively. All patients were examined with pre and post contrast MR imaging. Results The clinic symptoms included precocious puberty (n=5), gelastic seizure (n=3), other types of seizures (n=4), polydipsia and polyuria (n=1). MRI showed all masses were located in the suprasellar and interpeduncularis cistern regions with spheroidal or irregular shape .All masses were closely related to the tuber cinereum or the mammillary body, diameter ranging from 12mm to 25mm.The masses presented as iso-isointense to gray matter on T1-weighted imaging and T2-weighted imaging, or slightly high intense on T2- weighted imaging, without enhancement by gadolinium. Conclusion The clinical and MRI features of hypothalamic hamartomas are characteristic, which is helpful for diagnosis.