Neurofibromatosis 2

Abstract

THE neurofibromatoses consist of two distinct disorders,^{1 , 2} the genes for which have recently been located on separate chromosomes.^{3 4 5 6} Neurofibromatosis 1, also called von Recklinghausen's neurofibromatosis, affects approximately 100,000 people in the United States. It is a common and potentially serious inherited disorder of the human nervous system.¹ Neurofibromatosis 2, also known as bilateral acoustic neurofibromatosis, affects several thousand Americans and has a molecular origin similar to that of some of the common sporadically occurring tumors of the nervous system — meningiomas, schwannomas, neurofibromas, and possibly gliomas.³ This review discusses the clinical features, diagnostic advances, treatment dilemmas, and basic research findings . . .