下丘脑错构瘤的MR诊断与临床表现

MR diagnosis and clinical manifestation of hypothalamic hamartomas

目的:分析下丘脑错构瘤的MRI表现,探讨其临床表现、病理及MRI表现的关系,以提高对本病的认识。方法:回顾性分析了14例下丘脑错构瘤的MR及临床资料,其中男6例,女8例,年龄1～37岁。均进行了MR常规扫描和增强扫描,均经病理证实。结果:14例病灶中以宽基底附于第三脑室底部、灰结节和乳头体者6例,有"蒂"错构瘤6例,1例位于鞍背后方,1例异位于视交叉上方,所有病例均呈等T1WI信号,等或稍长T2WI信号,信号较均匀,边界较清晰。结节状或类圆形者9例,不规则形5例。直径<15mm者10例,>15mm者4例,最大直径2.9cm。2例有明显占位效应,向上压迫三脑室,向前压迫垂体柄或视交叉。结论:当患者出现性早熟和(或)痴笑样癫痫为主的临床表现,MRI显示灰结节和乳头体肿块并典型信号改变且无明显强化时,应首先考虑本病。

Objective： To study the MRI findings of ypothalamic hamartomas and evaluate the correlation of imaging features, pathological mechanism and clinical symptoms. Methods：MRI and clinical data of 14 patients （ included 6 boys and 8 girls, mean age 137 years） with hypothalamic hamartomas proved by operation and pathology were reviewed retrospectively. All patients were examined with pro-and post-contrast MR imaging; Results： All masses had homogeneous isointense with gray matter on T weighted imaging and isointensity or slight hyperintensity on T2 weighted imaging, with spheroidal （n=9） or irregular shape （n=5）. 6 cases had sessile attached to the floor of the third ventricle, tuber cinerea and mamillary body, 6 cases had pediele. 2 cases have obvious local mass effect. Conclusion： In children presenting with precocious puberty or gelastic epilepsy, masses of the tuber cinerea or mamillary bodies with MR signal characteristic mentioned above, the diagnosis of hypothalamic hamartoma should be considered.