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The effect of growth hormone treatment or physical training on motor performance in Prader-Willi syndrome: a
Linda Reus1, Leo A van Vlimmeren, J Bart Staal
1Radboud University Nijmegen Medical Centre, Department of Rehabilitation, Pediatric Physical Therapy, Nijmegen, The Netherlands; Radboud University Medical Centre, Scientific Institute for Quality of Healthcare, Nijmegen, The Netherlands. l.reus@reval.umcn.nl
Insights
Motor problems in Prader-Willi syndrome (PWS) significantly impact development from infancy through adulthood. Early intervention with growth hormone (GH) treatment and physical training shows promise for improving motor skills and overall development in PWS patients.
Area of Science:
- Pediatrics
- Endocrinology
- Neurology
- Physical Therapy
Background:
- Prader-Willi syndrome (PWS) is characterized by significant motor impairments affecting infants, children, and adults.
- Existing knowledge on factors influencing clinical management of PWS motor deficits is limited.
- Motor issues in PWS encompass developmental delays, reduced muscle force, impaired skill acquisition, and decreased physical fitness.
Purpose of the Study:
- To review and characterize the prevalence and nature of motor problems in Prader-Willi syndrome.
- To evaluate the impact of growth hormone (GH) treatment on motor performance in PWS.
- To assess the effects of physical training interventions on motor outcomes in individuals with PWS.
Main Methods:
- A systematic literature search was conducted to identify relevant studies.
- Included studies focused on motor performance, GH treatment, and physical training in PWS.
- Analysis synthesized findings from 34 identified research papers.
Main Results:
- Infants with PWS exhibit motor development at 30-57% of normal reference values.
- Growth hormone (GH) treatment demonstrated positive effects on motor performance across all age groups, though not universally.
- Physical training interventions consistently showed beneficial effects for motor function in PWS patients.
Conclusions:
- A combination of GH treatment and physical training is recommended for PWS patients, initiated as early as possible, particularly in infancy.
- Early intervention is crucial for improving motor development, which is expected to positively influence overall development in Prader-Willi syndrome.
- These combined therapies offer a promising strategy for managing and mitigating motor deficits associated with PWS.
Abstract:
Although motor problems in Prader-Willi syndrome (PWS) are prominent in infants, and continue into childhood and adulthood, there is little insight into the factors important for clinical management. The literature was reviewed to: (1) provide an overview of the characteristics and prevalence of motor problems and (2) evaluate the effects of growth hormone (GH) treatment and physical training on motor performance. A systematic search revealed 34 papers: 13 on motor performance; 12 on GH treatment; and nine on physical training. In infants, motor development is 30-57% of the normal reference values, and children and adults also have significant problems in skill acquisition, muscle force, cardiovascular fitness, and activity level. GH treatment positively influenced motor performance in infants, children, and adults, although not all studies demonstrated an effect. All studies on physical training demonstrated beneficial effects in PWS patients. We suggest a combination of GH treatment and physical training to be started as soon as possible, especially in infants, to improve motor development as this will positively influence general development.
