Cognition in Chiari Malformation Type I: an Update of a Systematic Review

Maitane García; Imanol Amayra; Manuel Pérez; Monika Salgueiro; Oscar Martínez; Juan Francisco López-Paz; Philip A Allen

doi:10.1007/s11065-023-09622-2

Cognition in Chiari Malformation Type I: an Update of a Systematic Review

Neuropsychol Rev. 2023 Oct 6. doi: 10.1007/s11065-023-09622-2. Online ahead of print.

Authors

Maitane García¹, Imanol Amayra², Manuel Pérez^{2

3}, Monika Salgueiro⁴, Oscar Martínez², Juan Francisco López-Paz², Philip A Allen⁵

Affiliations

¹ Department of Psychology, Faculty of Health Sciences, Neuro-E-Motion Research Team, University of Deusto, Bilbao, Spain. maitane.garciamartin@deusto.es.
² Department of Psychology, Faculty of Health Sciences, Neuro-E-Motion Research Team, University of Deusto, Bilbao, Spain.
³ Faculty of Health Sciences, Isabel I University, Burgos, Spain.
⁴ Department of Clinical and Health Psychology, and Research Methodology, Faculty of Psychology, University of the Basque Country, Donostia, Spain.
⁵ Conquer Chiari Research Center, Department of Psychology, University of Akron, Akron, OH, USA.

PMID: 37798373
DOI: 10.1007/s11065-023-09622-2

Abstract

Chiari malformation has been classified as a group of posterior cranial fossa disorders characterized by hindbrain herniation. Chiari malformation type I (CM-I) is the most common subtype, ranging from asymptomatic patients to those with severe disorders. Research about clinical manifestations or medical treatments is still growing, but cognitive functioning has been less explored. The aim of this systematic review is to update the literature search about cognitive deficits in CM-I patients. A literature search was performed through the following electronic databases: MEDLINE, PsychINFO, Pubmed, Cochrane Library, Scopus, and Web of Science. The date last searched was February 1, 2023. The inclusion criteria were as follows: (a) include pediatric or adult participants with a CM-I diagnosis, (b) include cognitive or neuropsychological assessment with standardized tests, (c) be published in English or Spanish, and (d) be empirical studies. Articles that did not report empirical data, textbooks and conference abstracts were excluded. After the screening, twenty-eight articles were included in this systematic review. From those, twenty-one articles were focused on adult samples and seven included pediatric patients. There is a great heterogeneity in the recruited samples, followed methodology and administered neurocognitive protocols. Cognitive functioning appears to be affected in CM-I patients, at least some aspects of attention, executive functions, visuospatial abilities, episodic memory, or processing speed. However, these results require careful interpretation due to the methodological limitations of the studies. Although it is difficult to draw a clear profile of cognitive deficits related to CM-I, the literature suggests that cognitive dysfunction may be a symptom of CM-I. This suggest that clinicians should include cognitive assessment in their diagnostic procedures used for CM-I. In summary, further research is needed to determine a well-defined cognitive profile related to CM-I, favoring a multidisciplinary approach of this disorder.

Keywords: Cerebellum; Chiari malformation type I; Cognition; Systematic review.

Publication types

Review