Taylor M J, Chan-Lui W Y, Logan W J
Can J Neurol Sci. 1985 May;12(2):100-5. doi: 10.1017/s0317167100046783.
We studied multimodal evoked potentials (EPs) longitudinally in a series of children with Friedreich's ataxia and ataxia telangiectasia to determine both their diagnostic utility and their correlation with clinical regression. The auditory brainstem responses (ABRs) were abnormal only in the children with Friedreich's ataxia. The abnormality seen in these patients was a rostral-caudal loss of the ABR waves. The visual EPs (VEPs) were abnormal in many of the patients; those with ataxia telangiectasia had unusually low amplitude or absent VEPs, occasionally with increased latencies, whereas those with Friedreich's ataxia had normal amplitude VEPs, often at increased latencies. The somatosensory EPs were usually of increased latency or absent in these patients. Unlike the ABR and VEPs, they did not serve to differentiate the groups. Changes in the EPs appeared to reflect clinical deterioration; patients with little change in their EPs over several years were regressing very slowly, whereas others had rapid deterioration in both EPs and clinical status. We suggest that the EPs are diagnostically of value in degenerative ataxias and may be of value in monitoring these patients and their response to therapy.
我们对一系列患有弗里德赖希共济失调和共济失调毛细血管扩张症的儿童进行了多模式诱发电位（EPs）的纵向研究，以确定其诊断效用及其与临床衰退的相关性。仅在患有弗里德赖希共济失调的儿童中，听觉脑干反应（ABRs）异常。这些患者中观察到的异常是ABR波从头部到尾部的缺失。许多患者的视觉诱发电位（VEPs）异常；患有共济失调毛细血管扩张症的患者VEPs振幅异常低或缺失，偶尔潜伏期延长，而患有弗里德赖希共济失调的患者VEPs振幅正常，但潜伏期常常延长。这些患者的体感诱发电位通常潜伏期延长或缺失。与ABR和VEP不同，它们无法区分这两组患者。诱发电位的变化似乎反映了临床恶化；在数年中诱发电位变化不大的患者临床衰退非常缓慢，而其他患者的诱发电位和临床状况都迅速恶化。我们认为，诱发电位在退行性共济失调的诊断中具有价值，并且在监测这些患者及其对治疗的反应方面可能具有价值。
