Prasher D, Bannister R
J Neurol Neurosurg Psychiatry. 1986 Mar;49(3):278-89. doi: 10.1136/jnnp.49.3.278.
Brain stem potentials from three groups of patients, namely those with pure progressive autonomic failure, Parkinson's disease and multisystem atrophy with progressive autonomic failure (Shy-Drager syndrome) were compared with each other and a group of normal subjects. In virtually all the patients with multisystem atrophy with progressive autonomic failure the brain stem potentials were abnormal in contrast to normal findings with Parkinson's disease. The closely associated group of patients with progressive autonomic failure alone also revealed no abnormalities of the BAEP. This separation of the two groups, Parkinson's disease and progressive autonomic failure from multisystem atrophy with progressive autonomic failure is important clinically as multiple system atrophy of the Shy-Drager type has extra-pyramidal features closely resembling Parkinsonism or a late onset cerebellar degeneration. From the abnormalities of the brain stem response in multisystem atrophy with progressive autonomic failure, it is clear that some disruption of the auditory pathway occurs in the ponto-medullary region as in nearly all patients there is a significant delay or reduction in the amplitude of components of the response generated beyond this region. The most likely area involved is the superior olivary complex.
对三组患者的脑干电位进行了相互比较，并与一组正常受试者进行了比较。这三组患者分别是患有单纯进行性自主神经功能衰竭的患者、帕金森病患者以及伴有进行性自主神经功能衰竭的多系统萎缩（夏伊-德雷格综合征）患者。实际上，几乎所有伴有进行性自主神经功能衰竭的多系统萎缩患者的脑干电位均异常，而帕金森病患者的检查结果正常。仅患有进行性自主神经功能衰竭的密切相关组患者的脑干听觉诱发电位（BAEP）也未显示异常。帕金森病组和进行性自主神经功能衰竭组与伴有进行性自主神经功能衰竭的多系统萎缩组的这种区分在临床上很重要，因为夏伊-德雷格型多系统萎缩具有与帕金森病极为相似的锥体外系特征或迟发性小脑变性。从伴有进行性自主神经功能衰竭的多系统萎缩患者脑干反应的异常情况来看，很明显听觉通路在脑桥延髓区域发生了某种中断，因为几乎所有患者在此区域之外产生的反应成分的幅度都有明显延迟或降低。最可能受累的区域是上橄榄复合体。
