View of the base and frontal lobes of the brain showing tubers or hard, potato like gyri at the arrows.
Tubers contain malformed neurons and astrocytes which form the foci for seizures.
The prevalance of tuberous sclerosis is 1 in 10,000 children ages 0-5. The face often shows angiofibromas on the cheeks and forehead, the heart may have rhabdomyomas and the kidneys may show cysts or angiomyolipomas. Microscopically the tuber is disorganized and contains large pyramidal like cells that have both neural and glial staining on immunoperioxidase as well as reactive gliosis. Candlegutterings contain similar cells which are most commanly glial in origin. The candleguttering may develop giant cell astrocytomas which are very slow growing but may cause death because they are difficult to remove.