Equilibrio: The effects of muscle hypotonia and weakness on balance: A study on Prader–Willi and Ehlers–Danlos syndrome patients

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Manuela Galli^{a, b}, Veronica Cimolin^{a, c, ,} , Luca Vismara^c, Graziano Grugni^d, Filippo Camerota^e, Claudia Celletti^e, Giorgio Albertini^b, Chiara Rigoldi^a and Paolo Capodaglio^c

^a Bioeng. Dept., Politecnico di Milano, p.zza Leonardo Da Vinci 32, 20133 Milano, Italy
^b IRCCS “San Raffaele Pisana”, Tosinvest Sanità, Roma, Italy
^c Orthopaedic Rehabilitation Unit and Clinical Lab for Gait Analysis and Posture, Ospedale San Giuseppe, Istituto Auxologico Italiano, IRCCS, Via Cadorna 90, I-28824 Piancavallo (VB), Italy
^d Unit of Auxology, Ospedale San Giuseppe, Istituto Auxologico Italiano, IRCCS, Via Cadorna 90, I-28824 Piancavallo (VB), Italy
^e Physical Medicine and Rehabilitation Division, Umberto I Hospital, Sapienza University, Piazza Aldo Moro 5, 00185 Rome, Italy

Received 17 December 2010; 

revised 10 January 2011; 

accepted 11 January 2011. 

Available online 9 February 2011.

Abstract

Prader–Willi syndrome (PWS) and Ehlers–Danlos syndrome (EDS) are two different genetical disorders both characterized, among other features, by muscular hypotonia. Postural control seems to be impaired in both conditions. The aim of the present study was to quantitatively compare postural control in adult PWS and EDS using stabilometric platform to unveil possible common determinants of impaired balance. We enrolled 11 PWS and 21 EDS adult patients and 20 age-matched controls. They were instructed to maintain an upright standing position for 30 s with open eyes (OEs) focusing on a 6 cm black circle positioned at a distance of 1.5 m.
Both PWS and EDS patients were characterized by higher RANGEML, RANGEAP and trajectory length of CoP values as compared to CG. No statistically differences were found between PWS and EDS in terms of any of these parameters. The results demonstrated that both PWS and EDS are characterized by a severe postural instability. Muscle hypotonia and weakness may account for reduced balance capacity. Quantitative characterization of instability is important to identify, develop and enhance rehabilitation interventions.

Research highlights

► Prader–Willi and Ehlers–Danlos syndrome are characterized by some common clinical and functional features. ► Prader–Willi and Down patients presented abnormal similar postural control. ► Both syndromes should be encouraged to undergo specific balance training.

Keywords: Prader–Willi; Ehlers–Danlos; Posture; Rehabilitation

Table 1. Clinical characteristics of the study groups (PWS: Prader–Willi syndrome; EDS: Ehlers–Danlos syndrome; CG: Control group). Data are expressed as mean (standard deviation).
⁺ p < 0.05, PWS group vs. EDS group.
^* p < 0.05, compared with Control group.

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Table 2. Postural parameters of the study groups (PWS: Prader–Willi syndrome; EDS: Ehlers–Danlos syndrome; CG: Control group). Data are expressed as mean values (standard deviation). The values are normalized for the subject's height and foot length.
^* p < 0.05 PWS and EDS group compared with CG.

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Corresponding author at: Bioeng. Dept, Politecnico di Milano, p.za Leonardo da Vinci 32, 20133 Milano, Italy. Tel.: +39 02 23993359; fax: +39 02 23993360. 

Fuente:

Research in Developmental Disabilities