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February 1969

Familial Central Nervous: Case Report of a Family With von Recklinghausen's Neurofibromatosis

Author Affiliations

Los Angeles
From the Departments of Medicine (Neurology) (Dr. Lee) and Surgery (Neuro-surgery) (Dr. Abbott), University of California, Los Angeles, School of Medicine.

Arch Neurol. 1969;20(2):154-160. doi:10.1001/archneur.1969.00480080054006

THE FAMILIAL occurrence of central nervous system (CNS) neoplasia is a rare event. Hauge and Harvald1,2 found no increased risk to relatives of propositi with glioma, meningioma, or medulloblastoma. Van der Wiel,3 however, found in his series of 100 patients with central glioma that 1 of 200 parents and 2 of 500 sibs were similarly affected; a risk he felt to be four times greater than expected. Several reviews4,5 have compiled isolated instances of cerebral tumors in twins and in blood relatives totaling about 50 reports in all. From these reports a genetic predisposition is suggested. The occurrence of a variety of CNS neoplasms associated with von Recklinghausen's disease (neurofibromatosis) has been documented in the literature.6 The purpose of this report is to describe a truly remarkable family in which eight members have been affected by CNS tumors. Only minor evidence of peripheral von Recklinghausen's

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