Novel Advances in Cognitive Impairmentin Amyotrophic Lateral Sclerosis

doi:10.12114/j.issn.1007-9572.2021.02.100

Abstract

Abstract:

Cognitive impairment is frequently found in patients with amyotrophic lateral sclerosis (ALS) , whose presence often suggests a poor prognosis.Recent years have seen some achievements in relevant research. We reviewed the clinical features, biomarkers, genetic features and latest therapeutic progress of cognitive impairment in ALS, aiming at providing new ideas for clinical management of this disease.

Key words: Amyotrophic lateral sclerosis, Cognitive dysfunction, Treatment, Review

摘要：

认知功能障碍在肌萎缩侧索硬化（ALS）患者中是普遍存在的，ALS伴认知功能障碍常提示预后不良。近年来对ALS患者认知功能障碍的研究取得了一定的进展，本文对ALS伴认知功能障碍的临床特点、生物标志物、遗传学特征和最新治疗进展进行综述，以期为ALS伴认知功能障碍的临床诊疗提供新思路。

关键词: 肌萎缩侧索硬化, 认知功能障碍, 治疗, 综述

Shuanghui GUO,Yumei ZHANG. Novel Advances in Cognitive Impairmentin Amyotrophic Lateral Sclerosis[J]. Chinese General Practice, 2022, 25(12): 1524-1528. DOI: 10.12114/j.issn.1007-9572.2021.02.100.
郭双辉,张玉梅. 肌萎缩侧索硬化伴认知功能障碍的研究进展[J]. 中国全科医学, 2022, 25(12): 1524-1528. DOI: 10.12114/j.issn.1007-9572.2021.02.100.

Figures/Tables 1

References 43

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突变基因	发生率（%）	病理蛋白	基因功能	遗传方式	FTD	其他临床表现
SOD1^[24]	25.3	SOD1	增强	AD、AR	否	感觉障碍、括约肌功能异常
TARDBP^[30，31]	5.8	TDP-43	缺陷	AD	是	帕金森样表现、进行性核上性麻痹
FUS^[2，30]	5.8	FUS	缺陷	AD	是	帕金森样表现
DCTN1^[32，33]	3.6	TDP-43	缺陷	AD	是	Perry综合征、进行性核上性麻痹、遗传性远端神经病
C9Orf72^[25]	3.5	TDP-43、p62、二肽重复蛋白	缺陷	AD	是	共济失调、亨廷顿病样表现、皮质基底节变性

突变基因	发生率（%）	病理蛋白	基因功能	遗传方式	FTD	其他临床表现
SOD1^[24]	25.3	SOD1	增强	AD、AR	否	感觉障碍、括约肌功能异常
TARDBP^[30，31]	5.8	TDP-43	缺陷	AD	是	帕金森样表现、进行性核上性麻痹
FUS^[2，30]	5.8	FUS	缺陷	AD	是	帕金森样表现
DCTN1^[32，33]	3.6	TDP-43	缺陷	AD	是	Perry综合征、进行性核上性麻痹、遗传性远端神经病
C9Orf72^[25]	3.5	TDP-43、p62、二肽重复蛋白	缺陷	AD	是	共济失调、亨廷顿病样表现、皮质基底节变性