【摘要】 目的 研究下丘脑神经元错构瘤的MR影像表现，并探讨其病理、临床及影像学的关系。方法 回顾性分析了7例下丘脑神经元错构瘤的MR影像资料。其中男3例、女4例，年龄2～14岁。6例进行了MR平扫和增强扫描，1例仅作了MR平扫。7例中，3例经手术、病理证实，4例为临床和影像学诊断。结果 全部病例在4岁前均出现临床症状，主要临床表现为性早熟和(或)痴笑样癫痫。其中6例在2岁前开始出现异常表现，3例在6个月前出现症状。T₁ WI上，所有病例均表现为均匀性、等信号强度影，肿瘤以广基与灰结节和乳头体相连。T₂ WI上，6例有性早熟的患儿中，3例肿块表现为高信号影，3例呈稍高信号影；1例单纯表现为痴笑样癫痫的病例，肿块呈等信号强度影。本组7例中，6例做了增强扫描，均未见肿块有强化表现。本组资料表明，临床表现与肿瘤大小及“蒂”无明显关系。结论 当患儿出现性早熟或痴笑样癫痫，MR显示灰结节和乳头体球形肿块伴典型信号改变，应首先考虑本病。本组资料表明，T₂ 信号的变化可能与分泌颗粒有一定关系，而肿瘤大小及“蒂”与临床表现无明显关系。

MR imaging of hypothalamic neuronal hamartomas

GAO Peiyi, LIN Yan. Neuroimaging Center, Beijing Neurosurgical Institute, Beijing 100050

【Abstract】 Objective To study the MRI findings of hypothalamic neuronal hamartomas and evaluate the correlation of imaging features and clinical symptoms. Methods MR study of 7 patients with hypothalamic neuronal hamartomas were reviewed retrospectively. The patients with precocious puberty and/or gelastic epilepsy, included four girls and three boys, ranging in age from 2 to 14 years. Six children were examined with precontrast and postcontrast T₁ -weighted MR imaging and with noncontrast T₂ -weighted MR imaging. Only one was scanned without contrast study. Three had surgery, with pathologic confirmation, and four were diagnosed by clinical and imaging features. Results Initial clinical symptoms were noted before the age of 4 years in all children, before the age of 2 years in six, and before the age of 6 months in three. MR study of all children demonstrated lesion with homogeneous signal intensity isointense with gray matter on T₁ -weighted MR images. All masses had sessile attachment to and originated from the tuber cinereum or mamillary bodies. The signal intensity of T₂ -weighted images was hyperintense relative to gray matter in 3 children with precocious puberty, slight hyperintense in 3 children with precocious puberty and isointense in one cases of isolated gelastic epilepsy. With contrast MR scan, there was no enhancement of the mass in all six cases. No correlation in the size or pedunculation of mass with clinical features was found. Conclusions In children presenting with precocious puberty or gelastic epilepsy, masses of the tuber cinereum or mamillary bodies with MR signal characteristics mentioned above, the diagnosis of hypothalamic neuronal hamartoma should be considered. Our data suggested that the signal intensity of the mass on T₂ -weighted images seemed to correlate with secretory granules. No correlation in the size or pedunculation of the mass with clinical features was found in our cases.

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