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Motor performance in children with Noonan syndrome
Ellen A Croonen1, Marlou Essink2, Ineke van der Burgt3
1Department of Pediatrics, Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, The Netherlands.
American Journal of Medical Genetics. Part A
|June 20, 2017
Summary
Children with Noonan syndrome show significant impairments in motor performance, strength, and endurance. These issues, including clumsiness and reduced muscle strength, impact daily functioning and may benefit from physical and occupational therapy.
Area of Science:
- Pediatric Neurology
- Developmental Pediatrics
- Clinical Motor Control
Background:
- Noonan syndrome is often associated with motor difficulties in daily life.
- A systematic investigation of the motor performance profile in affected children has been lacking.
Purpose of the Study:
- To systematically examine the motor performance profile in children with Noonan syndrome.
- To identify specific impairments using validated norm-referenced tests.
Main Methods:
- Assessed 19 children with Noonan syndrome using the Visual Motor Integration (VMI) test and Movement Assessment Battery for Children 2, Dutch version (MABC-2-NL).
- Measured grip strength, muscle force, and 6-minute Walking Test (6MWT) distance.
- Assessed for generalized hypermobility.
Main Results:
- Children with Noonan syndrome exhibited significantly lower standard scores on VMI and MABC-2-NL (except manual dexterity).
- Grip strength, muscle force, and 6MWT distance were significantly reduced; generalized hypermobility was more prevalent.
- Motor performance deficits correlated with reduced muscle strength and visual perception.
Conclusions:
- This study confirms significant impairments in motor performance, strength, and endurance in children with Noonan syndrome.
- Decreased functional motor performance appears linked to visual perception deficits and reduced muscle strength.
- Further research into causal relationships and intervention effectiveness is warranted; physical and occupational therapy may enhance daily life participation.
