OSTEOPATHIA STRIATA WITH CRANIAL SCLEROSIS - VARIABLE EXPRESSIVITY INA 4 GENERATION PEDIGREE

Osteopathia striata is a manifestation of several bone dysplasias. In association with cranial sclerosis it represents a separate entity, wh ich is not limited to the bones but may affect other structures, leadi ng to abnormal face, cleft palate, deafness, heart defects, and verteb ral anomalies. Neurological findings range from normal development to marked retardation with hydrocephalus, cranial nerve deficiencies and deafness, Ten families, including our own, clearly demonstrate autosom al dominant inheritance with female preponderance and great inter- and intrafamilial variability. (C) 1996 Wiley-Liss, Inc.