Pelosi L, Fels A, Petrillo A, Senatore R, Russo G, Lönegren K, Calace P, Caruso G
Acta Neurol Scand. 1984 Nov;70(5):360-8. doi: 10.1111/j.1600-0404.1984.tb00837.x.
In 15 patients definitely affected by Friedreich's ataxia, precocious SEP abnormalities were constantly recorded independent of the duration of symptoms and the severity of clinical involvement; VEPs were frequently involved, but neither VEP abnormalities, nor visual impairment could be correlated with the severity or duration of Friedreich's ataxia clinical involvement; BAEPs were, to different extents, abnormal. These were completely dissociated from hearing disorders and significantly correlated (P less than 0.001) with Friedreich's ataxia clinical disability. Our observation of increased latency of the VEP P100 component and the early disappearance of BAEP Wave V with persistence of Wave I is in contrast with the hypothesis that changes are related to primary axonal degeneration. For practical purposes SEPs could help in the early diagnosis of Friedreich's ataxia, and BAEPs could be used for the electrophysiological monitoring of its clinical progression.
在15例确诊患有弗里德赖希共济失调的患者中，始终记录到早期体感诱发电位（SEP）异常，且与症状持续时间和临床受累严重程度无关；视觉诱发电位（VEP）常受累，但VEP异常和视力损害均与弗里德赖希共济失调临床受累的严重程度或持续时间无关；脑干听觉诱发电位（BAEP）在不同程度上异常。这些异常与听力障碍完全无关，且与弗里德赖希共济失调的临床残疾程度显著相关（P<0.001）。我们观察到VEP的P100成分潜伏期延长以及BAEP的V波早期消失而I波持续存在，这与变化与原发性轴索变性有关的假设相反。从实际应用角度来看，SEP有助于弗里德赖希共济失调的早期诊断，而BAEP可用于其临床进展的电生理监测。
