Menor F, Martí-Bonmatí L, Mulas F, Poyatos C, Cortina H
Department of Diagnostic Radiology, Hospital Infantil La Fe, Valencia, Spain.
Pediatr Radiol. 1992;22(7):485-9. doi: 10.1007/BF02012989.
CT, MRI and neurological features of 27 children with tuberous sclerosis were prospectively compared. Imaging studies were positive in 92.5% of cases. CT was more useful in detecting subependymal nodules, while MRI showed the number and location of cerebral cortical and subcortical lesions more accurately. Cortical lesions in the cerebellar hemispheres were present in 26% of patients. Gadolinium-DTPA used in 10 patients showed slight enhancement of the subependymal lesions in 80% of cases, probably representing active lesions with alteration of the blood-brain barrier. None of the cortical and subcortical lesions enhanced. Giant-cell astrocytomas were detected in 5 patients, the postcontrast CT and MRI studies improving their assessment. Unlike subependymal nodules, all tumors showed marked enhancement regardless of their size. Seizures were present in 96% of patients, mostly beginning before 1 year of age. There was no clear relationship between the radiological features and the neurological evolution of these patients. Therefore, it is not possible to establish a clinical prognosis based on the radiological findings. MRI is the procedure of choice in the diagnosis of tuberous sclerosis. When pre and postcontrast MRI are negative, CT is used to exclude small calcified subependymal nodules. MRI follow-up is required only when tumoral development is clinically suspected.
对27例结节性硬化症患儿的CT、MRI及神经学特征进行了前瞻性比较。影像学检查在92.5%的病例中呈阳性。CT在检测室管膜下结节方面更有用，而MRI能更准确地显示大脑皮质和皮质下病变的数量及位置。26%的患者小脑半球存在皮质病变。10例患者使用钆喷酸葡胺后，80%的病例室管膜下病变有轻微强化，可能代表血脑屏障改变的活动性病变。皮质和皮质下病变均无强化。5例患者检测到巨细胞星形细胞瘤，增强CT和MRI检查有助于对其进行评估。与室管膜下结节不同，所有肿瘤无论大小均有明显强化。96%的患者有癫痫发作，大多在1岁前开始。这些患者的放射学特征与神经学进展之间没有明确关系。因此，不可能根据放射学表现来确定临床预后。MRI是诊断结节性硬化症的首选检查方法。当增强前后的MRI均为阴性时，使用CT排除小的钙化室管膜下结节。仅在临床怀疑有肿瘤进展时才需要进行MRI随访。
