Influence of Pain on Cognitive Dysfunction and Emotion Dysregulation in Chiari Malformation Type I

Abstract

It has been well demonstrated that the cerebellum is associated with cognitive and affective processing as well as the traditionally conceptualized motor function. In the present chapter, we explore the behavioral and neurobiological implications of a common congenital cerebellar condition, Chiari malformation Type I, on cognitive and affective processing. We also emphasize the associations between Chiari-related chronic pain, cognitive dysfunction, and emotion dysregulation. Based on our review of the literature, we argue that chronic pain can account for a substantial amount of the cognitive dysfunction and emotion dysregulation in Chiari malformation Type I. Yet, there also exists aspects of Chiari-related cognitive dysfunction and emotion dysregulation that appear to be at least partially independent of chronic pain and more directly associated with abnormalities in cerebrospinal fluid flow dynamics and cerebro-cerebellar communication pathways.

Keywords: Attention; Chiari malformation; Chronic pain; Cognitive control; Emotion regulation.

Fig. 11.1

Midsagittal radiological presentation of (a) a healthy control and (b) a patient diagnosed with Chiari malformation Type I. Image adapted from Houston et al. (2018b)

Fig. 11.2

Conceptual biopsychosocial model of pain and consequence on the quality of life. Chronic pain has reciprocal relationships with biological, psychological, and social functions, which have evocative interactions with each other and on health-related quality of life. Figure inspired by Duenas et al. (2016)

Fig. 11.3

Study protocol for the research that resulted in the publications of Houston et al. (2018b, , , . For more information on the self-report and behavioral measures, please see the following references: Short-Form McGill Pain Questionnaire (Melzack 1987); Depression Anxiety and Stress Scale (Henry and Crawford 2005), Neck Pain Disability Index Questionnaire (Fairbank et al. 1980), Chiari Symptom Profile (Mueller and Oro’ 2013), Repeatable Battery for the Assessment of Neuropsychological Statue-Update (RBANS) (Randolph 2012), Iowa Gambling Task (Bechara et al. 1994), and Facial Expression Identification tasks and electroencephalogram recording (Pollock et al. 2012)

Fig. 11.4

Depiction of a single trial of the PRP paradigm as used in Houston et al. (2018b). Participant responses to the second task are generally longer when there is a shorter lag between the two tasks. T1-Task 1; T2-Task 2; SOA-stimulus onset asynchrony between the two tasks. Figure adapted from Houston et al. (2018b)

Fig. 11.5

Electroencephalogram-based P3 event-related potentials by emotion across measured channel locations for CMI and control participants in Houston et al. (2018b). Facial expression stimulus onset is represented by 0 on the x-axis. Lines represent the three emotional expression conditions derived from the NimStim database. Differences in neurophysiological responses were apparent across emotion condition in controls at both midline central (Cz) and midline parietal (Pz) channels. CMI patients did not exhibit distinct patterns of neurophysiological activity in response to the different emotional expressions at any measured channel site. μV-microvolts; ms-milliseconds. Figure adapted from Houston et al. (2018b)

Fig. 11.6

Common morphometric measures in the Chiari malformation literature. Figure adapted from Houston et al. (2018a), which showed no significant relationship of these measures to self-reported chronic pain

Fig. 11.7

Clusters of significant positive correlation between fractional anisotropy and self-reported pain in a sample of CMI patients and age-, sex-, and education-matched controls. Figure adapted from Houston et al. (2020)

Fig. 11.8

Regions of CMI-related relative hypoconnectivity between cerebellar seeds and areas of connectivity. Figure adapted from Houston et al. (2021) and originally published under CC BY 4.0

Fig. 11.9

Regions of CMI-related relative hyperconnectivity between cerebellar seeds and areas of connectivity. Figure adapted from Houston et al. (2021) and originally published under CC BY 4.0