Sasaki S, Tsutsumi Y, Yamane K, Sakuma H, Maruyama S
Department of Neurology, Tokyo Women's Medical College, Japan.
Acta Neuropathol. 1992;84(2):211-5. doi: 10.1007/BF00311398.
We report an autopsy case of respirator-assisted long-survival sporadic amyotrophic lateral sclerosis (ALS) with extensive involvement extending beyond the motor system. The involved areas included the globus pallidus, subthalamic nucleus, red nucleus, substantia nigra, Clarke's column, intermediolateral nucleus, Onuf's (Onufrowicz) nucleus, middle root zone of the posterior column, and the spinocerebellar tract. There was no impairment of ocular movements. Such sporadic ALS cases with extensive involvement extending beyond the motor system may constitute a distinct subgroup of this disease that differs from long-survival ALS (with or without respirator support) in which only the motor system is affected.
我们报告一例接受呼吸机辅助且长期存活的散发性肌萎缩侧索硬化症（ALS）尸检病例，其受累范围广泛，超出了运动系统。受累区域包括苍白球、丘脑底核、红核、黑质、克拉克柱、中间外侧核、奥努夫（奥努夫罗维茨）核、后柱中间根区以及脊髓小脑束。眼球运动未受损害。这种受累范围广泛且超出运动系统的散发性ALS病例可能构成该疾病的一个独特亚组，与仅运动系统受累的长期存活ALS（无论有无呼吸机支持）不同。
