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May 1950

GARGOYLISM (LIPOCHONDRODYSTROPHY)A Study of Ten Cases, with Emphasis on the Formes Frustes of the Disease

Author Affiliations

THIELLS, N. Y.

From the Research Department, New York State Department of Mental Hygiene, Letchworth Village.

Arch NeurPsych. 1950;63(5):681-712. doi:10.1001/archneurpsyc.1950.02310230003001
Abstract

THE CONDITION known as gargoylism (lipochondrodystrophy, or the Hunter-Hurler syndrome) shows such striking clinical pathologic and etiologic features that it may be considered one of the best defined disease entities of all types of mental defect. Yet the study of this condition has not received the attention it deserves. To be sure, typical instances of the disease are easily recognized, and many have been reported in the literature; but cases with incomplete and less striking clinical manifestations often escape recognition. These formes frustes, which are perhaps more numerous than the typical forms of the disease, show interesting clinical and pathologic aspects.

It is the purpose of this paper to contribute to the knowledge of gargoylism by describing a series of 10 cases. In the first section, 3 typical instances of the complete form of the disease will be briefly reported and the main clinical features of the disease reviewed. The

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