DIABETIC EXOPHTHALMIC DYSOSTOSIS

Abstract

This unique symptom complex, beginning in childhood and characterized by the triad of diabetes insipidus, exophthalmos and disease of the membranous bones, is of special interest in its relation to the study of the central vegetative and endocrine mechanisms grouped in the parainfundibular portion of the hypencephalon. It assumes added importance because of its bearing on certain dysostoses, which until now have been regarded as separate entities, but which in all probability are allied forms; namely, the hereditary cleidocranial dysostosis of Marie and Sainton,¹and the familial craniofacial dysostosis described by Crouzon and recently studied by Debré and Petot.²More recently, the kinship was further extended to include the surprising relationship with the xanthomatous diathesis, which Rowland³attempted to identify as a lipoid disorder of the reticulo-endothelial system. A survey of the literature reveals fourteen other cases with the syndrome to be discussed, the first one described