Bermejo-Pareja Félix, Puertas-Martín Verónica
Head of the Neurology Department, University Hospital "12 de Octubre", Madrid, Spain ; Biomedical Research Network on Neurodegenerative Disorders (CIBERNED), Carlos III National Research Institute, Madrid, Spain ; Department of Biomedical Sciences (ANECA), Complutense University of Madrid, Spain.
Tremor Other Hyperkinet Mov (N Y). 2012;2. doi: 10.7916/D89W0D7W. Epub 2012 Sep 14.
The classical concept of essential tremor (ET) as a monosymptomatic tremorogenic disorder has been questioned in the last decade as new evidence has been described. Clinical, neuroimaging, and pathological studies have described a probable structural basis (mainly in cerebellum) and evidence that ET is associated with subtle clinical cerebellar deficits and several non-motor clinical manifestations, such as cognitive and mood disorders. We performed literature searches in Medline, ISI Web of Knowledge, and PsycInfo databases. The aim of this review is to describe cognitive deficits associated with ET. First, we present a brief history of ET cognitive disorders presented. Second, we describe several clinical cross-sectional series demonstrating that ET is associated with mild cognitive deficits of attention, executive functions, several types of memory (working memory, immediate, short term, delayed, and possibly others) and, mood disorders (depression). Recent neuroimaging studies favor a cerebellar basis for these cognitive deficits. Population-based surveys confirm that mild cognitive dysfunction is not limited to severe ET cases, the entire ET group, including mild and undiagnosed cases, can be affected. Cohort studies indicated that ET cognitive deficits could be progressive and that ET patients had an increased risk of dementia. The mood and cognitive deficits in ET are in agreement with cognitive affective cerebellar syndrome described in patients with cerebellar disorders. New evidence, mainly from functional (neuroimaging) and prospective clinical studies would further bolster recent descriptions of ET clinical manifestations.
在过去十年中,随着新证据的出现,原发性震颤(ET)作为一种单症状性震颤疾病的经典概念受到了质疑。临床、神经影像学和病理学研究描述了其可能的结构基础(主要在小脑),并有证据表明ET与轻微的临床小脑功能缺陷以及几种非运动性临床表现相关,如认知和情绪障碍。我们在Medline、ISI Web of Knowledge和PsycInfo数据库中进行了文献检索。本综述的目的是描述与ET相关的认知缺陷。首先,我们简要介绍ET认知障碍的历史。其次,我们描述了几个临床横断面系列研究,这些研究表明ET与注意力、执行功能、几种类型的记忆(工作记忆、即时、短期、延迟以及可能的其他记忆类型)的轻度认知缺陷以及情绪障碍(抑郁)有关。最近的神经影像学研究支持这些认知缺陷的小脑基础。基于人群的调查证实,轻度认知功能障碍并不局限于严重的ET病例,整个ET群体,包括轻度和未确诊的病例,都可能受到影响。队列研究表明,ET认知缺陷可能是进行性的,并且ET患者患痴呆症的风险增加。ET中的情绪和认知缺陷与小脑疾病患者中描述的认知情感小脑综合征一致。新的证据,主要来自功能性(神经影像学)和前瞻性临床研究,将进一步支持最近对ET临床表现的描述。