Tuberous Sclerosis

  • Etiology:
  • Imaging:
    — Brain – subcortical tubers with abnormal signal extending to ventricles along radial fibers, subependymal nodules, subependymal giant cell astrocytoma (SEGA) near Foramen of Monroe
    — Skin – angiofibroma
    — Cardiac – rhabdomyoma
    — Lungs – lymphangioleomyomatosis
    — GI – cysts in liver + pancreas
    — Renal – cysts, angiomyolipomas in 50%
    — Bone – bone islands

Radiology Cases of Tuberous Sclerosis

Radiology Cases of Cortical Tubers in Tuberous Sclerosis

MRI of tuberous sclerosis
Coronal FLAIR MRI images without contrast of the frontal (above left), middle (above right) and posterior (below) aspects of the brain shows multiple bilateral poorly defined hyperintense areas located just beneath the cortex of the brain.

Radiology Cases of Subependymal Nodules in Tuberous Sclerosis

MRI of tuberous sclerosis
Axial T2 MRI without contrast of the brain (left) shows three rounded low intensity subependymal lesions along the lateral aspect of the left lateral ventricle. Axial FLAIR MRI image without contrast of the brain (right) shows multiple bilateral poorly defined hyperintense areas located just beneath the cortex of the brain.

Radiology Cases of Angiomyolipoma in Tuberous Sclerosis

Axial CT without contrast of the abdomen shows the right kidney to be normal in size and to contain multiple small low density lesions that when measured demonstrate negative Hounsfield units. The left kidney, which is massively enlarged, also contains multiple small low density lesions as well as one extremely large low density lesion in its anterior aspect.