Sannou Institute of Psychiatry and Neurology, Tokyo, Japan.
Proc Jpn Acad Ser B Phys Biol Sci. 2013;89(6):270-80. doi: 10.2183/pjab.89.270.
The convulsions of the EL mouse (EL) were described by Imaizumi et al. in 1954 and were established as epilepsy by Suzuki in 1976. The EL mouse has been kept as an inbred strain and is considered one of the best animal models originated in Japan. The mode of inheritance is autosomal dominant, and environmental risk factors for seizure occurrence are hypothesised to contribute to the polygenic background. Paroxysmal activities in the EL brain arise from the parietal cortex (PCX) and are augmented in the hippocampus, demonstrated by electrophysiology and autoradiography using 2-deoxy glucose when clinical symptoms of seizures appeared. The neurons in the EL PCX, where GABA activity is lower than that of DDY PCX demonstrate increased excitability to proprioceptive sensory input. After repetitive seizure-provoking stimuli, seizures are more easily induced, eventually occurring spontaneously. This phenomenon of "abnormal plasticity" is also observed in the EEG, decreasing GABA activity, expression of the immediately early gene, and various biochemical and molecular processes. This phenomenon is similar to the learning or progressive process of certain neurological diseases.
1954 年，Imaizumi 等人描述了 EL 小鼠（EL）的惊厥现象，并于 1976 年将其确立为癫痫。EL 小鼠作为近交系被保存下来，被认为是源自日本的最佳动物模型之一。遗传方式为常染色体显性遗传，并且假设癫痫发作发生的环境风险因素促成了多基因背景。EL 大脑中的阵发性活动源于顶叶皮层（PCX），并且在海马体中得到增强，这通过在出现癫痫临床症状时使用 2-脱氧葡萄糖进行电生理学和放射自显影来证明。EL PCX 中的神经元中 GABA 活性低于 DDY PCX，对本体感觉输入表现出更高的兴奋性。在重复的引发癫痫刺激后，癫痫更容易被诱发，最终会自发发生。这种“异常可塑性”现象也在 EEG 中观察到，GABA 活性降低，即时早期基因的表达以及各种生化和分子过程减少。这种现象类似于某些神经疾病的学习或进行性过程。
