Sclerosteosis in old age

Abstract

Sclerosteosis is a rare, progressive disorder in which bone overgrowth causes facial distortion and cranial nerve dysfunction. The intracranial pressure usually becomes elevated and sudden death often occurs in adulthood. Servival into old age is unusual, but we have recently had the opportunity to investigate 2 elderly patients. Their clinical and radiographic features suggest that the rate of progression diminishes in later life, and that in exceptional circumstances survival into old age is possible.