Postural control abnormalities related to sleep deprivation in patients with Marfan Syndrome.
Postural control abnormalities related to sleep deprivation in patients with Marfan Syndrome. J Vestib Res. 2019 Nov 08;: Authors: Micarelli A, Viziano A, Lanzillotta A, Giorgino FM, Pisano C, Ruvolo G, Alessandrini M 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
ConclusionA survey of SDB in a sample of persons with MFS reveals not only a high burden of SDB but also that conventional screening instruments have utility if adapted appropriately. Future studies should validate the utility of these screening tools given concerns that SDB may contribute to progression of aortic pathology in MFS.
ABSTRACT Marfan ’ s (MFS) syndrome is characterized by a defect in the connective tissue, which affects multiple organic systems. Therefore, the management of these patients requires a multidisciplinary approach. This case reports the orthodontic-surgical treatment of a patient who presented both Marfan ’ s and obstructive sleep apnea syndrome. Patient had malocclusion, TMJ clicking, vertical maxillary excess, mandibular retrognathia, severe esthetic compromise, signs and symptoms of obstructive sleep apnea syndrome, as well as alterationsin joints, cardiovascular and respiratory systems. He was treated with bi...
ConclusionsOur study shows a high prevalence of OSA and a high prevalence of overweight in MFS patients. We found some trends between OSA and cardiovascular features but we could not establish a solid association. Our study, however might be underpowered, and a multicenter collaborative study could be very useful to answer some important open questions.
Aims: Obstructive sleep apnoea (OSA) is associated with significant haemodynamic and metabolic abnormalities during sleep. Previous studies have suggested an association between OSA and aortic dilatation in patients with hypertension and patients with Marfan syndrome. We hypothesised that OSA would be associated with increased aortic size in all patients irrespective of other risk factors.
Conditions: Sleep-disordered Breathing; Snoring Intervention: Device: CPAP Sponsors: Johns Hopkins University; The Marfan Foundation; National Institutes of Health (NIH) Recruiting
Study Objectives:Because of associated abnormalities affecting connective tissue in various organs including airways, hypermobility syndrome has been associated with high risk for the development sleep apnea. Ehlers-Danlos syndrome (EDS) and Marfan syndrome (MFS) represent the most common hypermobility syndromes; therefore, the purpose of this review was to examine the prevalence of obstructive sleep apnea (OSA) in these populations.Methods:All publications and poster presentations written in English found through August 2018 that describe the prevalence of sleep apnea among people with EDS or MFS were included.Results:A t...
To evaluate the relationship between the severity degree of OSA (apnea/hypopnea index AHI>1) and palatal area and volume, measured by 3D analysis of digital casts in Marfan children.