Prader–Willisyndrome(PWS)andEhlers–Danlossyndrome(EDS)aretwodifferentgeneticaldisordersbothcharacterized,amongotherfeatures,bymuscularhypotonia.Posturalcontrolseemstobeimpairedinbothconditions.Theaimofthepresentstudywasto quantitativelycompareposturalcontrolinadultPWSandEDSusingstabilometricplatformtounveilpossiblecommondeterminantsofimpairedbalance.Weenrolled11PWS and21EDSadultpatientsand20age-matchedcontrols.Theywereinstructedtomaintainanuprightstandingpositionfor30swithopeneyes(OEs)focusingona6cmblack circlepositionedatadistanceof1.5m.Both PWSandEDSpatientswerecharacterizedbyhigherRANGEML,RANGEAPandtrajectorylengthofCoPvaluesascomparedtoCG.NostatisticallydifferenceswerefoundbetweenPWSandEDSintermsofanyoftheseparameters.Theresultsdemonstratedthatboth PWSandEDSarecharacterizedbyasevereposturalinstability.Musclehypotoniaandweaknessmayaccountforreducedbalancecapacity.Quantitativecharacterizationofinstabilityisimportanttoidentify,developandenhancerehabilitationinterventions.
The effects of muscle hypotonia and weakness on balance: a study on Prader-Willi and Ehlers-Danlos syndrome patients
CELLETTI C;
2011-01-01
Abstract
Prader–Willisyndrome(PWS)andEhlers–Danlossyndrome(EDS)aretwodifferentgeneticaldisordersbothcharacterized,amongotherfeatures,bymuscularhypotonia.Posturalcontrolseemstobeimpairedinbothconditions.Theaimofthepresentstudywasto quantitativelycompareposturalcontrolinadultPWSandEDSusingstabilometricplatformtounveilpossiblecommondeterminantsofimpairedbalance.Weenrolled11PWS and21EDSadultpatientsand20age-matchedcontrols.Theywereinstructedtomaintainanuprightstandingpositionfor30swithopeneyes(OEs)focusingona6cmblack circlepositionedatadistanceof1.5m.Both PWSandEDSpatientswerecharacterizedbyhigherRANGEML,RANGEAPandtrajectorylengthofCoPvaluesascomparedtoCG.NostatisticallydifferenceswerefoundbetweenPWSandEDSintermsofanyoftheseparameters.Theresultsdemonstratedthatboth PWSandEDSarecharacterizedbyasevereposturalinstability.Musclehypotoniaandweaknessmayaccountforreducedbalancecapacity.Quantitativecharacterizationofinstabilityisimportanttoidentify,developandenhancerehabilitationinterventions.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
