Neurological Complications of Fibrous Dysplasia of the Skull

Abstract

FIBROUS dysplasia of bone is a disease of unknown etiology in which cellular fibrous tissue replaces normal bone. The process can involve a single bone (monostotic form) or be disseminated throughout the body (polyostotic form) in a continuum of severity.^1-5 Polyostotic fibrous dysplasia associated with cafe au lait spots and precocious puberty in girls has become known as Albright's syndrome.⁶ Skull involvement is estimated to occur in about 50% of cases of polyostotic fibrous dysplasia,^2,7 while it has been reported as accounting for 10% to 27% of all monostotic forms of the disease in various series.^7-9 The differential diagnosis, radiographic appearance, and pathological features of fibrous dysplasia of the skull have been previously described.^7,8,10 The purpose of this report is to survey the incidence, course, and therapy of neurological complications in 50 cases with skull involvement seen at the Columbia-Presbyterian Medical Center throughout the