The syndrome of cognitive impairment in amyotrophic lateral sclerosis: a population-based study
The syndrome of cognitive impairment in amyotrophic lateral sclerosis: a population-based study
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DOI：
10.1136/jnnp-2011-300188
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发表时间：
2012-01-01
影响因子：
11
通讯作者：
Hardiman, Orla
中科院分区：
文献类型：
--
作者：
Phukan, Julie;Elamin, Marwa;Hardiman, Orla
背景
尽管人们对此有相当大的兴趣，但肌萎缩侧索硬化症（ALS）中基于人群的认知障碍发生频率、临床特征及自然病程尚不清楚。
方法
作者对160例爱尔兰新发ALS患者和110例匹配的对照者进行了一项基于人群的前瞻性认知功能研究。进行家访以收集人口统计学和神经心理学数据。使用最近公布的共识标准以及基于执行和非执行认知过程的领域分类对患者进行分类。
结果
13.8%的患者符合额颞叶痴呆的Neary标准。此外，34.1%无痴呆证据的ALS患者符合最近公布的认知障碍共识标准。与健康对照者相比，无痴呆的ALS患者在语言和记忆领域出现障碍的频率明显更高。这些缺陷主要发生在执行功能障碍的患者中。14%的ALS患者有认知障碍但无执行功能障碍的证据，并且在近一半的队列（46.9%）中未检测到认知异常。
结论
在新诊断为ALS的患者中，约14%伴有共病性痴呆。在无痴呆证据的ALS患者中，超过40%存在认知障碍，主要但不完全是以执行功能障碍的形式。ALS中的认知障碍并非普遍特征，其表现可能比之前所认识的更具异质性。
Background Despite considerable interest, the population-based frequency, clinical characteristics and natural history of cognitive impairment in amyotrophic lateral sclerosis (ALS) are not known.Methodology The authors undertook a prospective population-based study of cognitive function in 160 incident Irish ALS patients and 110 matched controls. Home-based visits were conducted to collect demographic and neuropsychological data. Patients were classified using the recently published consensus criteria and by a domain-based classification of both executive and non-executive cognitive processes.Results 13.8% of patients fulfilled the Neary criteria for frontotemporal dementia. In addition, 34.1% of ALS patients without evidence of dementia fulfilled the recently published consensus criteria for cognitive impairment. Non-demented ALS patients had a significantly higher frequency of impairment in language and memory domains compared to healthy controls. These deficits occurred primarily in patients with executive dysfunction. 14% of ALS patients had evidence of cognitive impairment without executive dysfunction, and no cognitive abnormality was detected in almost half the cohort (46.9%).Conclusion Co-morbid dementia occurs in approximately 14% of patients with a new diagnosis of ALS. Cognitive impairment, predominantly but not exclusively in the form executive dysfunction, is present in more than 40% of ALS patients who have no evidence of dementia. Cognitive impairment in ALS is not a universal feature, and its manifestations may be more heterogeneous than previously recognised.