Central neurofibromatosis with bilateral acoustic neuroma
Central neurofibromatosis with bilateral acoustic neuroma
复制标题
中枢神经纤维瘤病伴双侧听神经瘤
作者：
W. Kanter;R. Eldridge;R. Fabricant;J. Allen;T. Koerber
神经纤维瘤病包括常见的“外周”形式和最近记录的“中枢”形式。我们描述了130例的中心形式，其中9种是个人研究的，15种是报告的。中枢神经纤维瘤病伴双侧听神经瘤是一种常染色体显性遗传病，发病年龄约为20岁，伴有轻度皮肤变化。用放射免疫法和放射受体法测定了3种中枢神经纤维瘤病患者血清中神经生长因子的含量。只有神经生长因子的抗原活性升高。相反，在周围神经纤维瘤病中，只有神经生长因子的功能活性被报道增加。中枢和外周形式的神经纤维瘤病是密切相关但离散的疾病，似乎有单独的神经生长因子活性的改变。
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.