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Abstract
Amyotrophic lateral sclerosis (ALS) is a progressive neurological disorder involving both upper motor neuron (UMN) and lower motor neuron (LMN), eventually leading to muscle atrophy and weakness, bulbar palsy, and respiratory failure. [...]we described writing errors in a Chinese-speaking patient with ALS-FTLD. No language disorder or psychotic symptoms were reported by her caregivers. Besides dysarthria, other salient neurological signs were moderate muscle wasting and weakness of her bilateral upper limbs, scoring 3/5 on proximal and 4/5 on distal muscles. Spontaneous potentials (fibrillations and positive sharp waves) and chronic denervation (motor unit potentials with increased duration and decreased motor unit recruitment) were recorded in all four regions (brainstem, cervical, thoracic, and lumbosacral spinal cords). {Figure 1}[INLINE:1] In neuropsychological tests, she scored 21/30 in Mini-Mental State Examination, 14/30 in Montreal Cognitive Assessment (version of Peking Union Medical College Hospital), and 12/72 in frontal behavioral inventory. Under the comprehensive cognitive evaluation system of Peking Union Medical College Hospital,[2] she showed impairment mainly in domains of executive function, memory, calculation, and abstract reasoning. [...]the reliability of writing errors in predicting dementia/aphasia in ALS still needed to be tested. [...]neuropsychological mechanism of agraphia might indicate the vulnerable cortical area of ALS. Department of Neurology, Peking Union Medical College and Hospital, Chinese Academy of Medical Sciences, Beijing 100730 Li-Ying Cui: Department of Neurology, Peking Union Medical College and Hospital, Chinese Academy of Medical Sciences, Beijing 100730; Neurosciences Center, Chinese Academy of Medical Sciences, Beijing 100730 Jing Gao: Department of Neurology, Peking Union Medical College and Hospital,...