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The radiology of sclerosteosis

Department of Human Genetics, Medical School, University of Cape Town, South Africa

B. J. Cremin, F.R.A.C.R., F.R.C.R.

Department of Radiology, Groote Schuur Hospital, Cape Town, South Africa

H. Hamersma, M.B., Ch.B., M.D.

University of Pretoria, South Africa

Sclerosteosis is an unusual disorder in which increased skeletal density is associated with abnormalities of bony modelling. The condition is progressive and complications arise due to cranial nerve involvement. Death from raised intracranial pressure occurs in early adulthood.

In order to define the range and extent of the bony abnormalities, the radiographic features of 18 affected individuals have been analysed. The recognition of characteristic changes permits diagnostic precision.

Received for publication March 1, 1976.