Nagao Masahiro
Department of Neurology, Tokyo Metropolitan Neurological Hospital.
Rinsho Shinkeigaku. 2013;53(11):1393-5. doi: 10.5692/clinicalneurol.53.1393.
In the subsets of amyotrohic lateral sclerosis (ALS), totally-locked in state (TLS) is shown as the result of marked progression of motor neuron degeneration. In TLS, patients are impossible to move any voluntary muscles. As the result, patients with TLS cannot communicate with any augmentative and alternative communication devices(AACD) at present. To find the AACD that enables for TLS to communicate, we examined the clinical character, brain MRI, SPECT and evoked potentials in TLS. Brain MRI showed marked brain atrophy including the brainstem, but the occipital lobe was spared. SPECT and visual evoked potentials (VEP) showed preserved physiological function of the occipital lobe in TLS. The results suggest that neuronal degeneration in TLS is not restricted to motor system, but that the visual pathways are spared. Patients with TLS may be possible to use AACD that utilize the visual pathway.
在肌萎缩侧索硬化症（ALS）的亚组中，完全闭锁状态（TLS）是运动神经元显著退化的结果。在TLS中，患者无法移动任何自主肌肉。因此，目前TLS患者无法使用任何辅助和替代沟通设备（AACD）。为了找到能让TLS患者进行沟通的AACD，我们检查了TLS患者的临床特征、脑部MRI、SPECT和诱发电位。脑部MRI显示包括脑干在内的脑部明显萎缩，但枕叶未受影响。SPECT和视觉诱发电位（VEP）显示TLS患者枕叶的生理功能保存完好。结果表明，TLS中的神经元退化并不局限于运动系统，视觉通路未受影响。TLS患者可能可以使用利用视觉通路的AACD。
