Impaired attention and cognitive deficits associated with pain and autonomic symptoms in hypermobile Ehlers-Danlos syndrome: a pilot study.

Abstract

Purpose: Patients with hypermobile Ehlers-Danlos syndrome (hEDS) frequently present with circulatory dysfunction, including postural orthostatic tachycardia syndrome (POTS), and cognitive impairments, leading to substantial disability and limitations in daily functioning. Few studies have examined attention, and concentration and associated conditions in hEDS. In this case-control study, we used a comprehensive cognitive test battery to assess whether cognitive performance is impaired in individuals with hEDS and depends on different body positions.

Methods: Twenty-nine patients and 29 healthy controls (HC) were enrolled. Baseline cognitive assessments included the Performance Scale of an intelligence test (LPS), Montreal Cognitive Assessment (cognitive deficit screening), and Test of Attentional Performance (TAP). The main cognitive tests for assessing the effects of body position were conducted in randomized conditions (supine, standing, and standing legs-crossed) and included the Stroop test, Corsi block-tapping test, Trail Making Test Part B, and Wechsler Memory Scale-revised.

Results: Compared to HC, patients with hEDS had higher intellectual performance (p < .050), but besides relevant comorbid conditions, also significantly impaired attention in the TAP (p < .010) and an impairment of executive function assessed by the Stroop test (p < .010) in the legs-crossed compared to the supine position.

Conclusion: Attention in the hEDS group was impaired compared to HC, and executive performance was dependent on body position. Individuals performed worse when standing legs-crossed. Hence, impaired proprioception as present in hEDS may, along with comorbid conditions such as pain, be a contributing factor affecting executive function.