Hayashi H, Kato S
Department of Neurology, Tokyo Metropolitan Neurological Hospital, Japan.
J Neurol Sci. 1989 Oct;93(1):19-35. doi: 10.1016/0022-510x(89)90158-5.
We report 4 cases of amyotrophic lateral sclerosis (ALS) in the totally locked-in state (TLS); 2 of the cases were autopsied. The clinical and pathological findings of ALS reported previously were essentially included in those studied here. In all cases reported in this paper, the voluntary muscles, including the oculomotor muscles, deteriorated rapidly, resulting in respiratory failure within 1.5 years. Successive observation of the 4 cases confirmed that ontogenetically old motor functions, including oculomotor functions, were preserved in the advanced stage, and that the main lesions in the oculomotor system were supranuclear. The 2 autopsied cases showed widespread pathological lesions other than those of the pyramidal tract and motoneurons commonly seen in ALS. We review these findings, and discuss them from an ontogenetical aspect in terms of the development of motor functions and anatomical myelination, and compare them with clinico-pathological findings of progressive supranuclear palsy (PSP) and anatomical structures of the supranuclear oculomotor system in monkeys.
我们报告了4例处于完全闭锁状态（TLS）的肌萎缩侧索硬化症（ALS）患者；其中2例进行了尸检。先前报道的ALS的临床和病理结果基本包含在本研究中。在本文报道的所有病例中，包括动眼肌在内的随意肌迅速恶化，在1.5年内导致呼吸衰竭。对这4例患者的连续观察证实，在疾病晚期，包括动眼功能在内的个体发生学上较古老的运动功能得以保留，并且动眼系统的主要病变位于核上。2例尸检病例显示出除了ALS中常见的锥体束和运动神经元病变之外的广泛病理病变。我们回顾这些发现，并从个体发生学角度就运动功能的发育和解剖学髓鞘形成进行讨论，并将它们与进行性核上性麻痹（PSP）的临床病理结果以及猴子核上动眼系统的解剖结构进行比较。
