Jeste D V, Barban L, Parisi J
Exp Neurol. 1984 Jul;85(1):78-86. doi: 10.1016/0014-4886(84)90162-6.
We studied, in a "blind" and quantitative fashion, the density of cerebellar Purkinje cells in 17 adult cases of Huntington's disease (HD), 17 patients with other movement disorders, 17 with schizophrenia, and 23 normal controls. There was a highly significant reduction in Purkinje cell density in HD compared with any of the other three groups. A much smaller difference in neuronal density between patients with other movement disorders and normal controls was barely significant. Eight of the 17 HD patients and only 1 of the other 57 subjects had Purkinje cell density less than 50% of the mean for the normal controls. The low density of Purkinje cells in HD could not be attributed to aging, seizures, or cause of death, nor was it merely a part of a generalized brain atrophy. The loss of large Purkinje cells suggests that the neuronal loss in HD may not be restricted to small and medium-size neurons.
我们以“盲法”和定量方式研究了17例成年亨廷顿舞蹈病（HD）患者、17例患有其他运动障碍的患者、17例精神分裂症患者以及23名正常对照者的小脑浦肯野细胞密度。与其他三组中的任何一组相比，HD患者的浦肯野细胞密度均有极显著降低。其他运动障碍患者与正常对照者之间神经元密度的差异小得多，几乎没有统计学意义。17例HD患者中有8例，而其他57名受试者中只有1例的浦肯野细胞密度低于正常对照者均值的50%。HD患者浦肯野细胞密度低不能归因于衰老、癫痫发作或死亡原因，也不仅仅是全脑萎缩的一部分。大浦肯野细胞的丢失表明，HD中的神经元丢失可能不限于中小型神经元。
