摘要
研究遗传性小脑共济失调的诱发电位变化。方法采用多种形式诱发电位，对３６例此类疾病的患者进行检测，并与３０～４０名健康者作对比。结果全部患者至少存在１种以上的诱发电位异常。磁刺激运动诱发电位（ＭＥＰ）、脑干听觉诱发电位（ＢＡＥＰ）及胫后神经与正中神经体感诱发电位（ｔＳＥＰ、ｍＳＥＰ）的异常率分别为８３．３％、８８．９％、８０．０％和６２．５％。不同类型小脑共济失调的诱发电位异常率不同，各型ＢＡＥＰ的异常率普遍较高，橄榄－桥脑－小脑萎缩患者的ＭＥＰ与遗传性痉挛性共济失调的ｔＳＥＰ异常率也很高。ＭＥＰ测试时，刺激皮质在患者中所记录到的双峰波、多相波以及波宽增加，表明皮质运动神经元的异常放电。
Objective The purpose of this study was to observe the changes of evoked potentials (EPs) in the patients with hereditary cerebellar ataxias. Methods Thirty six patients suffered from such diseases, including Marie ataxia (20 cases), late cortical cerebellar atrophy (LCCA, 9 cases) and olivo ponto cerebellar atrophy (OPCA, 7 cases) underwent investigations with multimodal EPs. The data obtained by any modality of the EPs in the patients were analyzed in comparison to those achieved in healthy persons matched in age and height (control group). Results It was found that all patients (100%) exhibited pathological changes in at least one of the neurophysiologic tests. The abnormal rates of the various investigations were as follows: The abnormal rate of motor evoked potentials to magnetic stimulation (MEP) consisted of 83.3%, that of brainstem auditory evoked potentials (BAEP): 88.9%, that of tibial nerve somatosensory evoked potentials (tSEP): 80.0% and that of median nerve evoked potentials (mSEP): 62.5%. However, the proportions of the abnormalities in each test were not necessarily identical concerning different patterns of cerebellar ataxias. Generally high percentages on BAEP test were seen in all patterns of ataxias with the range from 85.0% to 100%. In addition, a great majority of the patients with Marie ataxia showed abnormal changes on tSEP test (94.1%) and the patients with OPCA revealed exceedingly high abnormal rate on MEP test (100%). The waveforms of double peak and polyphasicity as well as increased duration occurred in the processes of the cerebral cortices stimulated. It was likely that the phenomena were due to extreme excitability of cortical motoneurons. Conclusion Multimodal EPs were much more sensitive than any of the single evoked potential test in determining the pathological conditions. The findings in regard to the changes of multimodal EPs should be considered a diagnostic base for the classification about the chronic cerebellar degenerations.
出处
《中华神经科杂志》
CAS
CSCD
1997年第3期169-173,共5页
Chinese Journal of Neurology
关键词
小脑共济失调
诱发电位
遗传性
共济失调
Spinocerebellar degeneration Hereditary diseases Evoked potentials
