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Oligophrenia in Combination with Congenital Ichthyosis and Spastic Disorders:

Robert B. Kugel, M.D.
AMA Arch Intern Med. 1958;101(3):675. doi:10.1001/archinte.1958.00260150163023.
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This monograph has to do with the description and the discussion of twenty-eight cases of a syndrome of low-grade oligophrenia, congenital ichthyosis, and spastic disorders of Little's disease type. The authors are describing what they believe to be a new syndrome. So far as they are concerned, there have been no other reported cases in the literature. Their cases are drawn almost entirely from Vasterbotten County, in the north of Sweden.

The descriptions made of these patients are vivid, and the detailed discussion of the clinical implications is well presented. The authors have made rather exhaustive genetic investigation of these families by tracing the incidence of this problem in families back through ten generations. This fact alone renders this study an impressive one, inasmuch as such investigation would very likely be impossible in many countries throughout the world.

The authors feel that this is a disease with a genetic causation


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