Résumé
Tuberous sclerosis is heredofamilial neurocutaneous syndrome, or phakomatosis, with multisystem involvement including the brain. The four major intracranial manifestarions [of TS are cortical tubers, white matter abnormalities, subependymal nodules, and subependymal giant cell astrocytoma. Eighteen patients with a clinical diagnosis of Tuberous sclerosis were examined by MR imaging. Their ages ranged from 3 months to 20 years. In 18 [100%] patients, MR examinations showed subependymal nodules, multiple peripheral gyral lesions consistent with cortical tubers were demonstrated in 14 [77%] patients. Subependymal nodules were best visualized on spin-echo images obtained with short repetition times [TR], although hypointesity within the nodules consistent with calcification was present in 15 patients and was most evident on long TR spin-echo images. Cortical tubers were more frequently demonstrated on long TR spin-echo images. White matter lesions were detected in 8 [44%] patients and subependymal giant cell astrocytoma in 4 [22%] patients. Computed tomographic scans were obtained for comparison in 15 patients. Intracranial calcification was more accurately diagnosed with CT. However, MR imaging was more sensitive than CT in detecting cortical tubers. Therefore MR may be better for screening family members and others in whom tuberous sclerosis is a possibility