Schrøder H D, Reske-Nielsen E
Clin Neuropathol. 1984 Sep-Oct;3(5):210-6.
Fourteen cases of amyotrophic lateral sclerosis (ALS) were investigated neuropathologically, emphazising the sacral spinal cord which contains Onuf's nucleus X. The nucleus innervates the pelvic sphincters. In two cases, small striated pelvic muscles were studied. No changes characteristic of ALS were observed in Onuf's nucleus X, not even in 8 cases in which other caudal motoneuron nuclei presented a severe loss of neurons. The striated sphincters proper demonstrated no signs of neurogenic atrophy in contrast to muscles in the limbs. The bulbo- and ischiocavernosus muscles, also supposedly innervated by Onuf's nucleus, were without pathological changes. Moreover, the latter two muscles were found to have a composition very similar to that of the sphincters. This indicates that a characteristic morphology of the nucleus X-innervated muscles exists. A review of the clinical records of all the cases revealed, that although 8 demonstrated severe involvement of the lower extremities, only one presented vesico-rectal dysfunction which could be ascribed to ALS. But even in this case, the pelvic closure mechanisms appeared to be intact. The preservation of continence in ALS is related to the neuropathological findings, and the observations are compared with previous neuropathological studies concerning Onuf's nucleus X as well with experimental studies including this nucleus. It is pointed out that structural and biochemical differences must exist between nucleus X neurons and other motoneurons.
对14例肌萎缩侧索硬化症（ALS）患者进行了神经病理学研究，重点关注含有Onuf核X的骶脊髓。该核支配盆腔括约肌。在2例患者中，对小的骨盆横纹肌进行了研究。在Onuf核X中未观察到ALS的特征性变化，即使在8例其他尾侧运动神经元核出现严重神经元丢失的病例中也是如此。与肢体肌肉相比，真正的横纹肌括约肌未显示神经源性萎缩的迹象。球海绵体肌和坐骨海绵体肌也推测由Onuf核支配，未出现病理变化。此外，发现后两种肌肉的组成与括约肌非常相似。这表明存在由核X支配的肌肉的特征形态。对所有病例的临床记录进行回顾发现，尽管8例患者下肢受累严重，但只有1例出现了可归因于ALS的膀胱直肠功能障碍。但即使在这种情况下，盆腔闭合机制似乎也完好无损。ALS患者的控尿能力与神经病理学发现有关，并将这些观察结果与先前关于Onuf核X的神经病理学研究以及包括该核的实验研究进行了比较。指出核X神经元与其他运动神经元之间必然存在结构和生化差异。
