Yoshida M, Murakami N, Hashizume Y, Itoh E, Takahashi A
Department of Neurology, Higashi Nagoya National Hospital.
Rinsho Shinkeigaku. 1992 Mar;32(3):259-65.
We reported two respirator-aided 10-year-survival male patients with sporadic amyotrophic lateral sclerosis (ALS). Their ages at onset were 54 and 52, clinical features common in both cases, the durations to tracheotomy 32 months and 27 months respectively, and the durations for respiratory support over 8 years in both cases. There were normal external ocular movement, normal pelvic sphincter function, normal sensory system and no bed sores throughout 10 years of illness. The brain weighted 1,295 g in case 1 and 1,430 g in case 2, being normal macroscopically. The spinal cord was slightly wasted with atrophic anterior roots. The histopathological study demonstrated the typical pattern of ALS in spite of the prolonged survival with artificial respiration. Neuronal loss and gliosis of the motor nuclei in the spinal cord and brainstem were more striking than those of the natural course. Neither hypertrophic astrocyte, central chromatolysis nor neuronophagia was observed. This finding suggested inactive condition of the degeneration. The pattern of white matter degeneration in the cord indicated some pallor of anterior and lateral columns, severe pallor of pyramidal tracts and intact posterior column and spinocerebellar tracts. The giant pyramidal cells in the precentral gyrus were lost in both cases. Pyramidal degeneration was traceable up to the cerebral peduncles in the case 2 and undetectable in that portion in the case 1. On the other hand, the extraocular muscle nuclei, the Onuf's nuclei, the dorsal motor nucleus of the vagus nerve, intermediolateral and Clarke's nuclei were relatively preserved, though no cell count was done in Clarke's nuclei and Onuf's nuclei.(ABSTRACT TRUNCATED AT 250 WORDS)
我们报告了两名接受呼吸机辅助、存活10年的散发性肌萎缩侧索硬化症（ALS）男性患者。他们的发病年龄分别为54岁和52岁，两例患者的临床特征相同，气管切开时间分别为32个月和27个月，两例患者的呼吸支持时间均超过8年。在整个10年病程中，患者的眼球外展运动正常、盆底括约肌功能正常、感觉系统正常且无褥疮。病例1的脑重为1295克，病例2的脑重为1430克，宏观上均正常。脊髓稍有萎缩，前根萎缩。尽管通过人工呼吸延长了生存期，但组织病理学研究仍显示出典型的ALS模式。脊髓和脑干运动核的神经元丢失和胶质细胞增生比自然病程更为明显。未观察到肥大的星形胶质细胞、中央染色质溶解或噬神经元现象。这一发现提示退变处于不活跃状态。脊髓白质退变模式显示前柱和侧柱有些苍白，锥体束严重苍白，后柱和脊髓小脑束完整。两例患者中央前回的巨大锥体细胞均缺失。病例2中锥体退变可追溯至大脑脚，病例1在该部位未检测到。另一方面，眼球外肌核、奥努夫核、迷走神经背运动核、中间外侧核和克拉克核相对保留，不过未对克拉克核和奥努夫核进行细胞计数。（摘要截断于250字）
