Marliani A F, Tampieri D, Melançon D, Ethier R, Berkovic S F, Andermann F
Department of Neuroradiology, Bellaria Hospital, Bologna, Italy.
Can Assoc Radiol J. 1991 Oct;42(5):335-9.
Hypothalamic hamartomas may cause a peculiar epileptic syndrome characterized by seizures of laughter and precocious puberty. Four mentally handicapped patients suffering from gelastic epilepsy were referred to our institution for investigation; three of them also presented with precocious puberty. In all four cases magnetic resonance imaging (MRI) revealed a space-occupying lesion of the hypothalamus that was considered to be a hamartoma. Biopsies were not performed. Hamartomas appear isodense in plain computed tomography scans, and they do not enhance. Such lesions display an isointense signal in T1-weighted magnetic resonance images and a hyperintense signal in proton density and T2-weighted images. MRI is the procedure of choice for detecting such lesions at the base of the brain.
下丘脑错构瘤可能导致一种特殊的癫痫综合征，其特征为笑性发作和性早熟。四名患有痴笑性癫痫的智力障碍患者被转至我院进行检查；其中三人还伴有性早熟。在所有四例病例中，磁共振成像（MRI）均显示下丘脑有占位性病变，考虑为错构瘤。未进行活检。错构瘤在普通计算机断层扫描中表现为等密度，且不强化。此类病变在T1加权磁共振图像中呈等信号，在质子密度和T2加权图像中呈高信号。MRI是检测脑底部此类病变的首选检查方法。
