Fuchino Yutaka, Nagao Masahiro, Katura Takusige, Bando Mitsuaki, Naito Masayoshi, Maki Atsushi, Nakamura Ken, Hayashi Hideaki, Koizumi Hideaki, Yoro Takeshi
Graduate School of Medical Sciences, Kitasato University, 1-15-1 Kitasato Sagamihara, Kanagawa 228-8555, Japan.
Neurosci Lett. 2008 Apr 18;435(2):85-9. doi: 10.1016/j.neulet.2008.01.046. Epub 2008 Mar 24.
Amyotrophic lateral sclerosis (ALS) is a motor neuron disease characterized by progressive degeneration of upper and lower motor neurons. Patients with ALS progressively lose their ability to control voluntary movements and occasionally enter the totally locked-in state (TLS), in which they cannot move any part of their bodies including the eyes. In this study, we clarified the preserved abilities and reorganization of the motor system of a 73-year-old patient with ALS in the TLS using optical topography, a recently developed extension of near-infrared spectroscopy. The patient performed four cognitive tasks: dichotic listening, covert singing, word fluency, and motor imagery. The bilateral prefrontal and bilateral sensorimotor areas were activated during the two language-related tasks (dichotic listening task and the word fluency), the right prefrontal and sensorimotor areas during the covert singing task, and the right prefrontal and dorsal sensorimotor areas during the motor imagery task. Contralateral sensorimotor activation was not observed in the motor imagery task. These results suggest that cognitive functions can be preserved in ALS in the TLS, with sensorimotor areas playing an important role.
肌萎缩侧索硬化症（ALS）是一种运动神经元疾病，其特征是上下运动神经元进行性退化。ALS患者逐渐丧失控制自主运动的能力，偶尔会进入完全闭锁状态（TLS），即他们无法移动身体的任何部位，包括眼睛。在本研究中，我们使用光学地形图（近红外光谱学的最新扩展技术）阐明了一名处于TLS的73岁ALS患者运动系统的保留能力和重组情况。该患者进行了四项认知任务：双耳分听、默读唱歌、词语流畅性和运动想象。在两项与语言相关的任务（双耳分听任务和词语流畅性任务）中，双侧前额叶和双侧感觉运动区被激活；在默读唱歌任务中，右侧前额叶和感觉运动区被激活；在运动想象任务中，右侧前额叶和背侧感觉运动区被激活。在运动想象任务中未观察到对侧感觉运动激活。这些结果表明，处于TLS的ALS患者的认知功能可以保留，感觉运动区发挥着重要作用。
