BILATERAL ACOUSTIC NEUROFIBROMAS
- 1 July 1940
- journal article
- other
- Published by American Medical Association (AMA) in Archives of Neurology & Psychiatry
- Vol. 44 (1) , 76-99
- https://doi.org/10.1001/archneurpsyc.1940.02280070084004
Abstract
In 1930, Gardner and Frazier1reported a family in which von Recklinghausen's disease, in the form of bilateral tumors of the acoustic nerve, had been transmitted as a dominant mendelian trait through five generations. At the time of the original report, 38 of the 217 members of the family were affected. Necropsy was performed on 2 members of the family and revealed bilateral acoustic neurofibromas. After the publication of the first report, symptoms of a tumor of the spinal cord developed in 1 of the affected members. This tumor was removed and likewise proved to be a neurofibroma; the case was subsequently reported by Gardner.2 The present report, the third concerning this family, deals chiefly with the pathologic material obtained from 4 members since the last publication.3One of these members (case 4) represents the only case thus far observed in which the disease has been recognizedKeywords
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