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Neurogenetics Section, IDB Intramural Research Program, National Institute of Neurological and Communicative Disorders and Stroke, National Institutes of Health, Bethesda, MD (Drs. Kanter, Eldridge, Allen, and Koerber). Albany Medical College, Alany, NY. (Dr. Kanter), the Laboratory of Viral Carcinogenesis, National Cancer Institute, National Institutes of Health, Bethesda, MD (Dr. Fabricant), the Louisiana State University Eye Center, New Orleans, LA (Dr. Fabricant), and the Memorial Sloan-Kettering Cancer Center, New York, NY (Dr. Allen).
Neurofibromatosis includes the common "peripheral" form and a recently documented "central" form. We describe the central form in 130 cases from 9 kindreds personally studied and 15 reported kindreds. Central neurofibromatosis with bilateral acoustic neuroma is an autosomal dominant disorder beginning about 20 years of age, accompanied by mild skin changes. In three kindreds with central neurofibromatosis, we measured nerve growth factor in serum by radioimmunoassay and radioreceptor assay. Only the antigenic activity of nerve growth factor was increased. In contrast, in peripheral neurofibromatosis, only the functional activity of nerve growth factor has been reported increased. Central and peripheral forms of neurofibromatosis are closely related but discrete diseases which appear to have separate alterations in nerve growth factor activity.
Address correspondence and reprint requests to Dr. Eldridge, Clinical Neurogenetics Studies, Neuroepidemiology Section, IRP, NINCDS, NIH, Federal Building, Room 904, Bethesda, MD 20205.
Accepted for publication April 2, 1979.
Presented in part at the 29th annual meeting of the American Academy of Neurology, Atlanta, April 1977.
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