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June 1929


Author Affiliations


From the Neurologic Service of Dr. Israel Strauss at the Mt. Sinai Hospital.

Arch NeurPsych. 1929;21(6):1402-1420. doi:10.1001/archneurpsyc.1929.02210240177010

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,1 and the familial craniofacial dysostosis described by Crouzon and recently studied by Debré and Petot.2 More recently, the kinship was further extended to include the surprising relationship with the xanthomatous diathesis, which Rowland3 attempted 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

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