Neuropsychological Function and the Relationship Between Subjective Cognition, Objective Cognition, and Symptoms in Hypermobile Ehlers–Danlos Syndrome

Introduction

Hypermobile Ehlers–Danlos syndrome (hEDS), a subtype of the Ehlers–Danlos syndromes, is a connective tissue disorder that is associated with a number of cognitive and psychological symptoms. Very little research has directly examined the neuropsychological functioning profile in hEDS, but some research has found associations of hypermobility with cognitive difficulties, psychological symptoms, and structural brain differences.

Methods

The current research compared a 12-matched-pairs sample of individuals with hEDS to healthy controls on a comprehensive neuropsychological test battery, as well as measures of mood, pain levels, fatigue, subjective cognition, and ability to perform social activities. Participants were matched for age, gender, and years of education. A second analysis was completed for a larger group of 18 participants with hEDS to examine associations of cognition and other symptoms with subjective cognition.

Results

Our results did not reveal significant cognitive differences between the two groups for most cognitive measures. However, individuals with hEDS had lower scores for delayed verbal memory recall. In the larger hEDS sample, correlations between subjective cognition, objective cognitive test performance, and other clinical variables revealed significant correlations between subjective cognition and visuospatial planning and construction, working memory, and set-shifting. Additionally, subjective cognition was associated with anxiety, depression, fatigue, pain, and the ability to participate in social activities.

Conclusion

We conclude that cognitive difficulties reported by individuals with hEDS are likely fluctuating and may correspond with fluctuating symptoms including dysautonomia, pain, and sleep difficulties. Furthermore, subjective cognition appears to be especially correlated with other related symptoms of mood, pain, and fatigue.