【摘要】 目的探讨MRI对下丘脑错构瘤的诊断价值,提高对下丘脑错构瘤的MRI表现和临床表现的认识。资料与方法回顾性分析8例下丘脑错构瘤的MRI和临床表现资料。其中男5例,女3例,年龄1.8~12岁。全部病例在3岁前均出现临床症状,其中2例在6个月前出现症状,临床表现为性早熟7例,痴笑样癫痫4例。8例均行MRI平扫和增强扫描。结果MRI表现为鞍上下丘脑区结节或肿块,直径为9~12mm,呈类圆形、不规则形,均以宽基底附于第三脑室底部、灰结节和乳头体,T1WI上与脑灰质信号一致,T2WI上呈稍高信号,信号均匀,增强后均未见肿块强化。结论下丘脑错构瘤是导致儿童性早熟的常见原因之一,MRI是目前诊断下丘脑错构瘤的最佳影像学方法。更多还原

【Abstract】 Objective To evaluate the diagnostic value of MRI for hypothalamic hamartomas, and to improve the understanding of its MR features and clinical findings.Materials and Methods MRI and clinical data of 8 patients （included 5 boys and 3 girl, mean age 1.8～12 years ） with hypothalamic hamartomas proved by operation and pathology were reviewed retrospectively. The clinic symptoms included precocious puberty （n=7） and/or gelastic epilepsy （n=4）. Initial clinical symptoms were noted before the age of 3 years in all children, before the age of 6 months in 2 children. All patients were examined with pre and post contrast MR imaging.Results MRI showed abnormal nodules or masses located in the suprasella and hypothalamic regions with spheroidal or irregular shape, diameter ranging from 9mm to 21mm. All mass had sessile attached to the basilar part of third ventricle, tuber cinerea and mamillary body. All masses had homogeneous isointensity with gray matter on T₁ weighted imaging and slight hyperintensity relative to gray matter on T₂ weighted imaging. On contrast MR scan, there was no enhancement in all cases. Conclusion The hypothalamic hamartoma is one of the important causes of precocious puberty in children, MRI is the optimal method for diagnosing hypothalamic hamartoma.更多还原