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- 2018
肌萎缩侧索硬化症临床特征及诊治进展
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Abstract:
摘要：肌萎缩侧索硬化症(ALS)是一种致死性神经系统变性病，病变主要累及皮质脊髓束、脑干和脊髓前角细胞。临床表现为逐渐进展的肌肉无力、萎缩、肌束震颤、延髓麻痹及锥体束损害，最终导致吞咽及呼吸困难而死亡，15%的ALS患者可以合并有额颞叶痴呆。本文简述了ALS临床特点、生存期和影响因素，并重点对近年来ALS的诊断标准、神经电生理诊断及治疗进展进行综述。强调本病的早期诊断非常重要，临床医生应该提高认识并给予合理的治疗。
ABSTRACT: Amyotrophic lateral sclerosis (ALS), a fatal neurodegenerative disease, mainly affects the corticospinal tract, brainstem and spinal cord anterior horn. Its clinical characteristics include progressive muscle weakness, atrophy, fasciculation, bulbar palsy and signs of pyramidal tract damage, which eventually lead to dysphagia and respiratory muscle weakness and death from respiratory failure. Frontotemporal dementia (FTD) occurs in approximately 15% of ALS patients. This article describes the clinical characteristics, survival time and influencing factors of ALS. It reviews the recent advances in diagnostic criteria, neurophysiological diagnosis and treatment of ALS. It is important to emphasize the early diagnosis of ALS. Clinicians should improve their understanding of the disease and provide proper treatment
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