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July 1951


Author Affiliations

From the Laboratories and the Ophthalmological Division of the Mount Sinai Hospital.

AMA Arch Ophthalmol. 1951;46(1):14-21. doi:10.1001/archopht.1951.01700020017003

THE SYNDROME of chondrodystrophic changes in the skeleton, corneal opacities, enlargement of the spleen, and mental deficiency has been known as Hurler's syndrome since 1919.1 The term gargoylism2 was introduced on account of the characteristic facies of the patient. The disease is also known as dysostosis multiplex, a term which refers to the skeletal features in cases of this disease.

Up to the present, approximately 50 cases of gargoylism have been reported, in 16 of which autopsies were performed and the organs studied. The present case, including a detailed pathological study, was published by Dr. Lotte Strauss in 1948.3 Strauss stated that the anatomic findings in Hurler's disease are varied and their interpretation is controversial. Generally speaking, there is a disturbance in tissue cells in many organs, in which an unknown substance is stored in the form of so-called foam cells. According to Strauss, the involvement of

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