Assessment of physical function in adults with Prader-Willi syndrome

Abstract

Purpose. To evaluate implementation of at test battery for assessing physical function in adults with Prader-Willi syndrome (PWS).

Method. Forty-three adults with PWS, 20 men and 23 women, mean age 29 ± 9 years. Body mass index (BMI) and body composition were measured. A test battery a.m. Guralnik for standing balance, 2.4 m and 10 m walk and repeated rising from a chair was scored and evaluated.

Results. Mean BMI was 29.4 ± 9.1 kg/m², body fat mass (FM) 26.0 ± 16.1 kg. The adults with PWS managed the test battery well, and high scores were achieved for standing balance and 2.4 m walk. In contrast, only 13 managed maximum score in the repeated rising from a chair and 31 to walk 10 m for 6 s or faster. Significant correlations were seen between 2.4 m walk and FM ( p = 0.041) and between 10 m walk and BMI ( p = 0.001) and FM ( p = 0.019).

Conclusions. Repeated rising from a chair and 10 m walk are useful assessments in clinical practice to identify adults with PWS with reduced physical function. Reduced performance was related to higher BMI and FM emphasising the importance of efforts to keep normal body weight and body composition in adults with PWS. These measures might be useful in monitoring effects of intervention.

Keywords:

• PWS
• adults
• physical function
• assessment
• Guralnik