Tuberus Sclerosis is a multisystemic neuroectodermal disorder. It's incidence is between 1 in 6000 and 1 in 30,000. This disorder has a wide variety of expressions with a variable prognosis. There are numerous major and minor features and diagnosis depends on 2 major features or 1 major and 2 minor features. Approximately half of the cases are inherited in an autosomal dominant pattern, and the other half are sporadic. Classically, the clinical presentation "zits, fits, nit-wits"--adenoma sebacum, seizures and mental retardation.

This systemic disorder affects many systems. In the brain, it causes hamartomas in subependymal and subcortical (the Tubers) locations. The tubers may calcify, and may have enhancement on MRI. The subependymal hamartomas are most commonly located near the Foramen of Monroe. Additionally these patients are predisposed to Subependymal giant cell astrocytoma.

About half of these patients will have renal angiomyolipomas (fatty containg masses). These are usually bilateral and multiple. 50% will have numerous bone islands, periosteal thickening and or bone cysts. Less common associations are lymphangioleiomyomatosis, chylothorax, and cardiac rhabdomyomas.

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