Postural control abnormalities related to sleep deprivation in patients with Marfan Syndrome

Abstract

BACKGROUND: Marfan syndrome (MFS) is a rare autosomal dominant connective tissue disorder affecting virtually every organ. Sleep disturbances, associated to high collapsibility in upper airways, are common in MFS; daytime sleepiness could lead to reduction in attention and motor coordination, with detrimental effects on balance.OBJECTIVE: To evaluate otoneurological function in MFS patients, compared to healthy subjects, and to investigate possible correlations with sleep deprivation extent.METHODS: Forty-one MFS patients underwent a thorough otoneurological examination, video Head Impulse Test (vHIT), and static posturography. Sleep parameters were recorded by home monitoring. Daytime sleepiness and dizziness-related handicap were screened by means of Dizziness Handicap Inventory (DHI) and Epworth Sleepiness Scale (ESS). Results were compared with 49 healthy controls (HC).RESULTS: DHI and ESS scores were increased in MFS patients (p < 0,01). vHIT scores showed no between-group effect. Classical (surface and length) and frequency-domain posturographic parameters were significantly increased in MFS with respect to HC (p < 0 ,01). A positive correlation was found between ESS scores and posturographic parameters in MFS patients.CONCLUSIONS: An impaired postural control, related to the extent of sleep deprivation, was found in MFS patients. Such results could advocate for screening and treating sleep deprivation and balance dysfunctions in MFS patients.