Displacement of the cerebellar tonsils in Chiari type I malformation (CMI) can affect functions controlled by the cerebellum and brainstem. While playing an integral role in the control of movement, the cerebellum also has widespread cortical connections, influencing a range of cognitive process. A systematic literature review was conducted to examine the relationship between cognition and CMI, assessing evidence for general or domain-specific cognitive change. The search protocol examined the AMED, CINAHL, Cochrane Library, EMBASE, MEDLINE, PsycINFO, and Scopus databases. Articles meeting the following criteria were included in this review (i) examined children or adults with a clinically defined diagnosis of CMI, (ii) assessed cognitive function with a prospective examination, (iii) included at least one standardized instrument designed to measure general or specific domains of cognitive function, and (iv) were published in English in a peer-reviewed journal. Twelve articles were identified, including 783 cases aged 3 months to 64 years. General cognition, processing speed, and learning and memory appeared less affected, while language deficits appeared to diminish with age. Executive dysfunction was the most commonly reported cognitive impairment, while attention and working memory, and visuospatial and perceptual skills also appeared vulnerable. Numerous methodological limitations were identified that should be considered in interpreting the impact of CMI and planning future investigations. Overall, there is currently insufficient evidence to describe a valid and reliable profile of cognitive impairment in CMI. Further research is required to confirm these preliminary psychometric results and integrate them with pathophysiological models

中文翻译：

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对Chiari I畸形认知的系统评价

Chiari I型畸形（CMI）小脑扁桃体移位可能影响小脑和脑干控制的功能。小脑在控制运动中起着不可或缺的作用，同时也具有广泛的皮质连接，影响一系列认知过程。进行了系统的文献综述，以检查认知与CMI之间的关系，评估一般或特定领域认知变化的证据。搜索协议检查了AMED，CINAHL，Cochrane库，EMBASE，MEDLINE，PsycINFO和Scopus数据库。符合以下条件的文章包括在本评价中：（i）对具有临床定义的CMI诊断的儿童或成人进行了检查，（ii）通过前瞻性检查评估了认知功能，（iii）至少包括一种旨在测量认知功能的一般或特定领域的标准化工具，并且（iv）在同行评审期刊上以英文发表。确定了12篇文章，其中783例年龄在3个月至64岁之间。一般的认知，处理速度以及学习和记忆似乎受到的影响较小，而语言缺陷则随着年龄的增加而减少。执行功能障碍是最常报告的认知障碍，而注意力和工作记忆以及视觉空间和知觉技能也似乎很脆弱。在解释CMI的影响和计划未来的调查时，应确定许多方法上的局限性。总体而言，目前尚无足够的证据来描述CMI中认知障碍的有效和可靠概况。