Tsutsui J, Kawashima S, Kajikawa I, Shirabe T, Terao A
Doc Ophthalmol. 1986 Jun 16;63(1):13-21. doi: 10.1007/BF00153007.
In a case of Creutzfeldt-Jakob disease in a 59-year-old female, multifocal degeneration from the cerebral cortex through the visual pathway to the retina was detected clinically, electrophysiologically, and pathologically. Visual evoked cortical potentials (VECPs) showed a peculiar huge negative wave in the early stage but the amplitude reduced gradually. The a- and b-waves of the ERG were detectable in the final stage. Dynamic topography of VECPs revealed a delay of excitation in the visual cortex in the early stage, but a complete defect of the cortical potential and diminished reactivity of the brain stem were apparent in the later stage. Pathological findings were the spongy degeneration of the cerebral cortex, demyelination of the white matter and the optic pathway, and the degeneration of the nerve fiber layer and ganglion cell layer of the retina.
在一名59岁女性的克雅氏病病例中，临床上、电生理上和病理上均检测到从大脑皮质经视觉通路至视网膜的多灶性变性。视觉诱发电位（VECP）在早期显示出一个奇特的巨大负波，但波幅逐渐降低。视网膜电图的a波和b波在最后阶段仍可检测到。VECP的动态地形图显示早期视觉皮质兴奋延迟，但后期皮质电位完全缺失且脑干反应性降低。病理结果为大脑皮质海绵状变性、白质和视路脱髓鞘以及视网膜神经纤维层和神经节细胞层变性。
