Fukutani Y, Nakamura I, Kobayashi K, Yamaguchi N, Matsubara R
Division of Neuropsychiatry, National Sanatorium Hokuriku Hospital, Toyama, Japan.
J Neurol Sci. 1992 Mar;108(1):39-47. doi: 10.1016/0022-510x(92)90186-o.
Cerebellar dentate and interpositus nuclei pathology was studied morphometrically in 3 patients with Joseph disease compared to 3 control subjects. Size of neurons, number of small neurons (cell body area: 50-199 microns 2) and large neurons (cell body area: 200 microns 2 or greater) at the rostral, medial and caudal levels, neuronal cell density, total volume of the gray bands, and total neuronal cell number were evaluated in the dentate, emboliform, and globose nuclei, using an image analyzer, after making horizontal serial 20-microns thick sections of a unilateral cerebellar hemisphere embedded in celloidin. The number of large neurons in Joseph disease was around 20% of that in the controls at each level in the individual nucleus (P less than 0.05 or P less than 0.01). In contrast, the number of small neurons was significantly reduced only at the caudal level of the dentate nucleus (P less than 0.05). The neuronal cell density was decreased within the nuclei. The total volume of the gray bands was reduced to about 70% within the nuclei (P less than 0.05 or P less than 0.01). The total number of neurons was decreased to about a third, a half, and a third within the dentate (P less than 0.001), emboliform (P less than 0.01), and globose nuclei (P less than 0.001), respectively. The principal pathologic change of the dentate and interpositus nuclei in Joseph disease was severe loss of neurons, with significant loss of the large neurons, indicating that Joseph disease is a type of cerebellar efferent system disorder.
对3例约瑟夫病患者和3名对照者的小脑齿状核和间位核病理进行了形态计量学研究。在将单侧小脑半球包埋于火棉胶中制成20微米厚的水平连续切片后，使用图像分析仪评估齿状核、栓状核和球状核中神经元的大小、在嘴侧、中间和尾侧水平的小神经元（细胞体面积：50 - 199平方微米）和大神经元（细胞体面积：200平方微米或更大）数量、神经元细胞密度、灰质带的总体积以及神经元总数。在每个核的各个水平，约瑟夫病患者的大神经元数量约为对照者的20%（P < 0.05或P < 0.01）。相比之下，仅在齿状核的尾侧水平小神经元数量显著减少（P < 0.05）。核内神经元细胞密度降低。核内灰质带的总体积减少至约70%（P < 0.05或P < 0.01）。齿状核（P < 0.001）、栓状核（P < 0.01）和球状核（P < 0.001）内的神经元总数分别减少至约三分之一、二分之一和三分之一。约瑟夫病中齿状核和间位核的主要病理变化是神经元严重缺失，大神经元显著丢失，表明约瑟夫病是一种小脑传出系统疾病。
