Ochikubo F, Gomi H, Kitamura K, Yoshikawa Y, Yamanouchi K
Laboratory Animal Research Center, University of Tokyo, Japan.
Electroencephalogr Clin Neurophysiol. 1992 Feb;82(2):145-51. doi: 10.1016/0013-4694(92)90158-e.
Zitter rats with genetic spongiform encephalopathy and hypomyelination developed an abnormal auditory brain-stem response (ABR) before the appearance of spongy lesions in the central nervous system (CNS). The ABR abnormalities were characterized by a dual peak of wave I, with a longer latency than in normal rats, and decreased or absent waves III and IV. Hypomyelination in both peripheral and central nerves may have been responsible for these abnormalities. The slow negative wave became wide and obscure with aging. These changes accompanied age-dependent progression of spongy changes in the CNS. These findings suggest that at least two mechanisms, one involving hypomyelination and the other causing spongy lesions, are responsible for the brain-stem auditory pathway dysfunction in zitter rats.
患有遗传性海绵状脑病和髓鞘形成不足的颤抖鼠在中枢神经系统（CNS）出现海绵状病变之前就出现了异常的听觉脑干反应（ABR）。ABR异常的特征是I波出现双峰，潜伏期比正常大鼠长，且III波和IV波减弱或消失。外周和中枢神经的髓鞘形成不足可能是这些异常的原因。随着年龄增长，慢负波变得宽大且模糊。这些变化伴随着CNS中海绵状变化的年龄依赖性进展。这些发现表明，至少有两种机制导致了颤抖鼠的脑干听觉通路功能障碍，一种涉及髓鞘形成不足，另一种导致海绵状病变。
