Davis S L, Aminoff M J, Berg B O
Arch Neurol. 1985 Feb;42(2):156-60. doi: 10.1001/archneur.1985.04060020070019.
Brain-stem auditory evoked potentials (BAEPs) were recorded in 23 children who had signs of brain-stem or cerebellar dysfunction. In patients with brain-stem gliomas, BAEPs were abnormal in all except one, in whom involvement of the brain-stem auditory pathway was limited to the midbrain tectum. The BAEPs were normal in neuronal ceroid lipofuscinosis, but abnormal bilaterally in inheritable leukoencephalopathies. All patients with Leigh's encephalopathy had BAEP abnormalities; in two, abnormalities occurred before the appearance of lesions on computed tomographic scan. Patients with Friedreich's ataxia and giant axonal dystrophy had abnormal BAEPs, but the test was normal in a child with similar neurologic findings with vitamin E deficiency. Patients with diffuse metabolic encephalopathies had variable findings. Thus, BAEP abnormalities are nonspecific for various disease processes but are frequently seen in neoplastic and neurodegenerative diseases, with primary white matter or extensive brain-stem involvement.
对23名有脑干或小脑功能障碍体征的儿童进行了脑干听觉诱发电位（BAEP）记录。在脑干胶质瘤患者中，除1例脑干听觉通路受累仅限于中脑顶盖的患者外，其余患者的BAEP均异常。神经元蜡样脂褐质沉积症患者的BAEP正常，但遗传性白质脑病患者的BAEP双侧异常。所有 Leigh 脑病患者的BAEP均异常；其中2例在计算机断层扫描出现病变之前就出现了异常。弗里德赖希共济失调和巨大轴索性营养不良患者的BAEP异常，但在一名有类似神经系统表现的维生素E缺乏症儿童中，该检查结果正常。弥漫性代谢性脑病患者的检查结果各异。因此，BAEP异常对各种疾病过程而言并非特异性表现，但在肿瘤性和神经退行性疾病中常见，这些疾病伴有原发性白质受累或广泛的脑干受累。
