Ferrer I, Kulisevski J, Vazquez J, Gonzalez G, Pineda M
Department of Pathological Anatomy, Hospital Principes de España, Hospitalet de LLobregat, Barcelona, Spain.
Clin Neuropathol. 1988 Jan-Feb;7(1):22-8.
The cellular pathology of Purkinje cells in several degenerative diseases of the cerebellum and its connections was studied with the rapid Golgi method. Purkinje cells from a patient with Creutzfeldt-Jakob disease (case 1) showed decreased numbers of spiny branchlets. In a patient with a cystic infarct of the cerebellar white matter and chronic deafferentation of the cerebellar cortex (case 2) there was a striking increase in the density of spines on the primary dendrites. No abnormalities were observed in Purkinje cells from two patients with hereditary spinocerebellar degeneration (cases 3 and 4). Purkinje cells in two patients with olivopontocerebellar atrophy (OPCA) had severely reduced numbers of dendritic branches. Ballooned proximal axons were found in Purkinje cells with severely damaged dendrites (cases 5 and 6). In contrast to experimental olivary degeneration in laboratory animals, Purkinje cells from patients with OPCA did not have increased numbers of stubby spines in stout proximal dendrites but few spines with long pedicles were observed in the proximal segment of the primary dendrites and in the cellular body.
采用快速高尔基法研究了小脑几种退行性疾病及其联系中浦肯野细胞的细胞病理学。克雅氏病患者（病例1）的浦肯野细胞棘状小分支数量减少。小脑白质囊性梗死和小脑皮质慢性传入神经阻滞患者（病例2）的初级树突棘密度显著增加。两名遗传性脊髓小脑变性患者（病例3和4）的浦肯野细胞未观察到异常。两名橄榄体脑桥小脑萎缩（OPCA）患者的浦肯野细胞树突分支数量严重减少。在树突严重受损的浦肯野细胞中发现近端轴突呈气球样（病例5和6）。与实验动物的实验性橄榄体变性不同，OPCA患者的浦肯野细胞在粗壮的近端树突中没有增加短粗棘的数量，但在初级树突近端段和细胞体中观察到很少有带长蒂的棘。
