CONGENITAL DEFECTS OF THE CENTRAL NERVOUS SYSTEM ASSOCIATED WITH HYPERENDEMIC GOITER IN A NEOLITHIC HIGHLAND SOCIETY OF NETHERLANDS NEW GUINEA

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Abstract
A recently discovered, isolated, and still neolithic population in the interior Highlands of Netherlands New Guinea has been studied, in which most, and perhaps all, of the members suffer from varying degrees of congenital central nervous system damage. This damage ranges from subtle degrees of mental subnormality to severe feeble-mindedness often associated with deafness or deaf-mutism. In this region about one-fourth of the males and two-thirds of the females are found to have goiter. In some hamlets the incidence reaches 100% among the adult females. Many of these goiters are among the largest reported in world literature. The extreme isolation and the high incidence of this congenital goitrous cretinism of varying degrees of severity make this region ideally suited for studies of both the genetic and environmental factors which determine the defects in this population. Similarly, they offer an unprecedented opportunity to investigate the etiology and pathogenesis of central nervous system defect associated with endemic goiter. The possibility that an unusual use of sweet potato leaves as a dietary staple in a region wherein the total nutritional balance is marginally adequate may play a role in the medical situation is under investigation. Metabolic and biochemical studies will be reported in subsequent papers in this series. Other medical problems of the area are briefly discussed and the distribution of goitrous areas in New Guinea is reviewed.

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