Abstract

BackgroundImproving balance and reducing risk of falls is a relevant issue in Prader-Willi Syndrome (PWS). The present study aims to quantify the effect of a mixed training program on balance in patients with PWS.MethodsEleven adult PWS patients (mean age: 33.8 ± 4.3 years; mean BMI: 43.3 ± 5.9 Kg/m2) attended a 2-week training program including balance exercises during their hospital stay. At discharge, Group 1 (6 patients) continued the same exercises at home for 6 months, while Group 2 (5 patients) quitted the program. In both groups, a low-calorie, well-balanced diet of 1.200 kcal/day was advised. They were assessed at admission (PRE), after 2 weeks (POST1) and at 6-month (POST2). The assessment consisted of a clinical examination, video recording and 60-second postural evaluation on a force platform. Range of center of pressure (CoP) displacement in the antero-posterior direction (RANGEAP index) and the medio-lateral direction (RANGEML index) and its total trajectory length were computed.ResultsAt POST1, no significant changes in all of the postural parameters were observed. At completion of the home program (POST2), the postural assessment did not reveal significant modifications. No changes in BMI were observed in PWS at POST2.ConclusionsOur results showed that a long-term mixed, but predominantly home-based training on PWS individuals was not effective in improving balance capacity. Possible causes of the lack of effectiveness of our intervention include lack of training specificity, an inadequate dose of exercise, an underestimation of the neural and sensory component in planning rehabilitation exercise and failed body weight reduction during the training. Also, the physiology of balance instability in these patients may possibly compose a complex puzzle not affected by our exercise training, mainly targeting muscle weakness.

Highlights

  • Improving balance and reducing risk of falls is a relevant issue in Prader-Willi Syndrome (PWS)

  • In a previous study [15], we demonstrated that PWS patients have a poorer balance capacity than their nongenetically obese counterparts and our findings suggested that strengthening of ankle flexors/extensors, balance training and tailored exercises aimed at improving medial-lateral control using hip strategies should be given particular consideration within rehabilitation programs

  • PWS patients are characterized by an increase in BMI which is demonstrated to be associated with an increase in functional impairment [9], which could lead to impaired balance and an increased risk of falls

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Summary

Introduction

Improving balance and reducing risk of falls is a relevant issue in Prader-Willi Syndrome (PWS). Hyperphagia and weight gain between the ages of 1 and 6, lead most PWS patients to develop morbid obesity, affecting the development of motor and functional skills [3]. An increase in BMI is associated with an increase in functional impairment [9], which could lead to impaired balance and an increased risk of falls than normal-weight individuals under daily postural stresses and perturbations [10,11], even in younger individuals, under 40 years of age [12,13]. Obese individuals may fear falling, which may lead to further reductions in physical activity [8], greater functional impairment [14], and greater risk of falling

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