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特发性震颤与认知障碍:谁、如何以及为何。

Essential tremor and cognitive impairment: who, how, and why.

机构信息

Department of Clinical and Experimental Medicine, University of Messina, Messina, Italy.

"Rita Levi Montalcini" Department of Neuroscience, University of Turin, Turin, Italy.

出版信息

Neurol Sci. 2022 Jul;43(7):4133-4143. doi: 10.1007/s10072-022-06037-4. Epub 2022 Mar 31.

Abstract

BACKGROUND AND AIMS

Recent years have witnessed the switch from considering essential tremor (ET) a monosymptomatic disorder to consider it as part of a spectrum, including other neurological signs, such as mild cognitive impairment and dementia, thus defining it as "ET plus." There are few data on cognitive impairment in ET patients. The aim of this review is to analyze the clinical characteristics of ET patients developing cognitive impairment, their neuropsychological profile, the underpinning mechanisms, and the possible biomarkers.

METHODS

The authors performed a narrative review on cognitive decline in essential tremor, including articles written in English since the year 2000.

DISCUSSION

The most recent pathogenetic theories of cognitive impairment in ET rely on the cerebellar dysfunction, being part of the Cerebellar Cognitive Affective Syndrome spectrum. Cognitive impairment in ET patients could be assessed through many tests that demonstrate the involvement of different domains, such as attention, executive functions, and language. There are some clinical characteristics of ET that may indicate a greater risk of developing cognitive impairment, namely, cerebellar symptoms, falls, age at onset, and family history. However, there are no established clinical, neurophysiological, neuropathological, and fluid biomarkers of cognitive impairment in ET.

CONCLUSIONS

Increasing data are showing in ET the presence of cerebellar symptoms and cognitive impairment. Further studies are needed to better understand cognition in ET patients, and to define the boundary between ET and ET plus, since deeper phenotyping might have important clinical and therapeutic implications.

摘要

背景与目的

近年来,人们已不再将特发性震颤(ET)视为单一症状性疾病,而是将其视为一种谱系疾病,其中包括其他神经系统症状,如轻度认知障碍和痴呆,从而将其定义为“ET 伴发”。目前关于 ET 患者认知障碍的数据较少。本文旨在分析出现认知障碍的 ET 患者的临床特征、神经心理学特征、潜在机制和可能的生物标志物。

方法

作者对 2000 年以来发表的关于 ET 认知衰退的英文文章进行了综述。

讨论

ET 认知障碍的最新发病机制理论依赖于小脑功能障碍,属于小脑认知情感综合征谱。可通过许多测试评估 ET 患者的认知障碍,这些测试可证明不同领域的参与,如注意力、执行功能和语言。ET 患者存在一些可能预示认知障碍风险更高的临床特征,即小脑症状、跌倒、发病年龄和家族史。然而,目前尚无 ET 认知障碍的明确临床、神经生理学、神经病理学和液相关生物标志物。

结论

越来越多的数据表明 ET 患者存在小脑症状和认知障碍。需要进一步研究以更好地了解 ET 患者的认知情况,并定义 ET 和 ET 伴发之间的界限,因为更深入的表型分析可能具有重要的临床和治疗意义。

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