We describe a patient with sporadic amyotrophic lateral sclerosis (ALS) who showed progressive deterioration of sensory cortex excitability at the advanced stage, while using invasive ventilation. At the time of diagnosis, the patient showed enlarged N20 of the median nerve somatosensory evoked potential (SEP). Following ventilator use through tracheostomy, the patient gradually fell into a totally locked-in state for four years and the N20 showed progressive deterioration in the amplitude, which finally led to its loss. Magnetic resonance imaging (MRI) showed frontotemporal and mild parietal cortex atrophy, subcortical white matter hyperintensity and brainstem atrophy suggesting the involvement of the central sensory pathways. MRI and flash visual evoked potentials revealed that the occipital lobe was well-preserved throughout the course of the disease. This is the first case report of a physiological demonstration of multisystem neurodegeneration involving the central sensory pathway in a patient with advanced ALS and invasive ventilation use.

中文翻译：

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晚期肌萎缩性侧索硬化伴有创通气的感觉皮质兴奋性逐渐恶化。

我们描述了患有散发性肌萎缩性侧索硬化症（ALS）的患者，该患者在使用有创通气的同时，在晚期显示出感觉皮层兴奋性的逐渐恶化。在诊断时，患者显示正中神经躯体感觉诱发电位（SEP）的N20增大。在通过气管切开术使用呼吸机后，患者逐渐陷入完全锁定状态达四年之久，N20的振幅逐渐恶化，最终导致其丢失。磁共振成像（MRI）显示额颞叶和轻度顶叶皮质萎缩，皮层下白质高信号和脑干萎缩，提示中枢感觉通路的参与。MRI和闪光视觉诱发电位显示，在整个疾病过程中枕叶都保存完好。这是多发性神经退行性变的生理学演示的首例病例报告，涉及患有晚期ALS和有创通气的患者的中央感觉通路。