Dickson D W, Horoupian D S, Thal L J, Davies P, Walkley S, Terry R D
Neurology. 1986 Oct;36(10):1323-9. doi: 10.1212/wnl.36.10.1323.
We studied a patient with amyotrophic lateral sclerosis and the Klüver-Bucy syndrome. At autopsy there was extensive degeneration of the limbic system with the brunt of the changes in the medial temporal lobe, especially the entorhinal cortex and subiculum. Degenerative changes were also seen in the substantia nigra and lower motor neurons. Morphometric and biochemical studies implied a disease process that affected small, possibly somatostatinergic, cortical neurons. These latter findings and the lobar distribution of cortical atrophy were consistent with Pick's disease, but Pick bodies and ballooned neurons were not present.
我们研究了一名患有肌萎缩侧索硬化症和克吕弗-布西综合征的患者。尸检发现边缘系统广泛变性，内侧颞叶变化最为明显，尤其是内嗅皮质和海马下托。黑质和下运动神经元也出现了退行性变化。形态学和生化研究提示存在一种影响小型、可能为生长抑素能的皮质神经元的疾病过程。这些后期发现以及皮质萎缩的叶性分布与匹克病相符，但未发现匹克小体和气球样神经元。
