Neuropsychology Review

, Volume 28, Issue 2, pp 176–187 | Cite as

A Systematic Review of Cognition in Chiari I Malformation

  • Jeffrey M. RogersEmail author
  • Greg Savage
  • Marcus A. Stoodley


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


Arnold Chiari malformation Cognition Neuropsychological test Systematic review 



cerebellar cognitive affective syndrome


Chiari type I malformation


cerebrospinal fluid



This work was supported by a grant from the Column of Hope.

Compliance with Ethical Standards

Conflict of Interest

The authors have no conflicts of interest to report.


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© Springer Science+Business Media, LLC, part of Springer Nature 2018

Authors and Affiliations

  • Jeffrey M. Rogers
    • 1
    Email author
  • Greg Savage
    • 2
  • Marcus A. Stoodley
    • 1
  1. 1.Department of Clinical Medicine, Faculty of Medicine and Health SciencesMacquarie UniversityNew South WalesAustralia
  2. 2.ARC Centre of Excellence in Cognition and its Disorders, Department of PsychologyMacquarie UniversityNew South WalesAustralia

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