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March 1946

NEUROFIBROMATOSIS WITH DEFECT IN WALL OF ORBIT: Report of Five Cases

Author Affiliations

MINNEAPOLIS

From the Division of Neurosurgery, Department of Surgery, University of Minnesota Medical School.

Arch NeurPsych. 1946;55(3):248-265. doi:10.1001/archneurpsyc.1946.02300140079005
Abstract

SINCE von Recklinghausen described neurofibromatosis in 1882 there has been a radical change in the concept of this disease. It is now well recognized that the cutaneous tumors and the pigmentation, as described by von Recklinghausen, are only two manifestations of a congenital defect which, in its widespread involvement, may affect practically any or all of the systems of the body.

HEREDITY  The hereditary nature of neurofibromatosis has been conclusively established, but Preiser and Davenport1 have shown especially well that its inheritance follows regular mendelian principles and that the hereditary factor controlling it is a dominant one. They collected 30 cases from the literature in which 2 or more members of a family were affected. Uhlmann and Grossman2 examined 60 members of 3 families with Recklinghausen's disease and found evidence of the disease (either tumor formation or pigmentation) in 13 of these persons. In 1 family members of

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