April 1968

Neurological Complications of Fibrous Dysplasia of the Skull

Author Affiliations

New York
From the Neurological Clinical Research Center, Neurological Institute, Columbia-Presbyterian Medical Center, and Department of Neurology, College of Physicians and Surgeons, Columbia University, New York.

Arch Neurol. 1968;18(4):363-369. doi:10.1001/archneur.1968.00470340049004

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

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