Kihira T, Yoshida S, Yoshimasu F, Wakayama I, Yase Y
Division of Neurological Diseases, Wakayama Medical College, Japan.
J Neurol Sci. 1997 Mar 20;147(1):81-8. doi: 10.1016/s0022-510x(96)05313-0.
The Onufrowicz (Onuf's) nuclei from eight amyotrophic lateral sclerosis (ALS) cases and nine neurological control cases were studied histologically and morphometrically. To clarify the factors relating to the involvement of the Onuf's nucleus in ALS, we correlated the relationships among the age at death, clinical duration, morphometric findings for Onuf's neurons, and neuronal numbers in the posteroposterolateral (PPL) nuclei in the ALS cases with those in neurological controls. Intracytoplasmic inclusions such as Bunina bodies, ubiquitin-reactive inclusions, and conglomerate inclusion were found in the Onuf's neurons in ALS, but not in the controls. The total number of Onuf's neurons in the ALS cases was not decreased, but that of normal-appearing neurons was decreased while that of atrophic neurons was increased. Significantly decreased perikaryal, nuclear and nucleolar areas and decreased perikaryal (P)/nuclear and P/nucleolar area ratios of Onuf's neurons were found in ALS, not only in the atrophic neurons but also in the normal-appearing neurons, compared with the controls. The shrinkage in Onuf's neurons of ALS was different from that seen in the ageing process or in the axonal reactions of controls with atonic bladder. In ALS, the morphometric findings for the Onuf's neurons showed no correlation with age at death, clinical duration, or number of PPL neurons. Our results indicate that in ALS Onuf's nucleus is principally vulnerable to the ALS process, although the degree of degeneration differs from that seen in other motor neurons. The involvement of Onuf's nucleus might be slowed due to factors specific to this nucleus, including the biochemical and autonomic properties of the nucleus; nevertheless, it is histologically classified as part of the somatic cell column.
对8例肌萎缩侧索硬化症（ALS）患者和9例神经学对照患者的奥努夫核进行了组织学和形态计量学研究。为了阐明与奥努夫核在ALS中受累相关的因素，我们将ALS患者中奥努夫神经元的形态计量学结果、后外侧（PPL）核中的神经元数量与神经学对照患者的死亡年龄、临床病程进行了相关性分析。在ALS患者的奥努夫神经元中发现了胞质内包涵体，如布尼纳小体、泛素反应性包涵体和聚集性包涵体，但在对照中未发现。ALS患者奥努夫神经元的总数没有减少，但外观正常的神经元数量减少，而萎缩神经元数量增加。与对照相比，在ALS患者中，不仅萎缩神经元，而且外观正常的神经元，都发现奥努夫神经元的胞体、细胞核和核仁面积显著减小，胞体（P）/细胞核和P/核仁面积比降低。ALS患者奥努夫神经元的萎缩与衰老过程或张力性膀胱对照患者的轴突反应中所见的萎缩不同。在ALS中，奥努夫神经元的形态计量学结果与死亡年龄、临床病程或PPL神经元数量均无相关性。我们的结果表明，在ALS中，奥努夫核主要易受ALS进程影响，尽管其退变程度与其他运动神经元不同。由于该核的特定因素，包括其生化和自主神经特性，奥努夫核的受累可能会减缓；然而，在组织学上它被归类为躯体细胞柱的一部分。
