Motor Function of Children with SMA1 and SMA2 Depends on the Neck and Trunk Muscle Strength, Deformation of the Spine, and the Range of Motion in the Limb Joints
The purpose of this study was to investigate the functional relationships between selected ranges of motion of the neck, upper and lower limbs, the strength of the neck and trunk muscles, postural parameters, and the motor function of children with SMA1 and SMA2—27 children, aged 6 months-15 years,...
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doaj-f17db710763a4500bcad6c677e7218262021-09-09T13:45:11ZengMDPI AGInternational Journal of Environmental Research and Public Health1661-78271660-46012021-08-01189134913410.3390/ijerph18179134Motor Function of Children with SMA1 and SMA2 Depends on the Neck and Trunk Muscle Strength, Deformation of the Spine, and the Range of Motion in the Limb JointsAgnieszka Stępień0Ewa Gajewska1Witold Rekowski2Department of Rehabilitation, Józef Piłsudski University of Physical Education, Marymoncka Str., 00-968 Warszawa, PolandDepartment of Developmental Neurology, Poznan University of Medical Sciences, 49 Przybyszewskiego Str, 60-355 Poznan, PolandDepartment of Rehabilitation, Józef Piłsudski University of Physical Education, Marymoncka Str., 00-968 Warszawa, PolandThe purpose of this study was to investigate the functional relationships between selected ranges of motion of the neck, upper and lower limbs, the strength of the neck and trunk muscles, postural parameters, and the motor function of children with SMA1 and SMA2—27 children, aged 6 months-15 years, with genetically confirmed spinal muscular atrophy type 1 (19 children) and 2 (8 children) undergoing pharmacological treatment. All children were examined, according to the methodology, including the motor function evaluation, measurement of selected ranges of motion, assessment of postural parameters, and measurement of neck and trunk muscle strength. The functional status of 15 children was assessed with the CHOP INTEND (CHOP group) scale and of 12 children with the HFMSE (HFMSE group). The results obtained showed that, in children examined with the CHOP scale, greater limitation of flexion in the shoulder joints was observed. As the deformation of the chest increased, the functional abilities of children deteriorated. In participants examined with the CHOP group, the ranges of neck rotation decreased with the increase of the chest deformity. In the HFMSE group, the ranges of head rotation showed a strong relationship with some parameters of muscle strength and the sum of the R coefficients. Participants showed many significant relationships between the range of motion in the neck and joints of the limbs, with more significant relationships in the CHOP group. The following conclusions were made: motor skills of children with SMA depend on muscle strength, range of motion, and deformities of the spine and chest; the development of scoliosis adversely affects the motor function, ranges of motion, and muscle strength; and movement ranges are related to motor skills and strength values.https://www.mdpi.com/1660-4601/18/17/9134spinal muscular atrophymotor functionscoliosisrange of motionphysiotherapyrehabilitation |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Agnieszka Stępień Ewa Gajewska Witold Rekowski |
spellingShingle |
Agnieszka Stępień Ewa Gajewska Witold Rekowski Motor Function of Children with SMA1 and SMA2 Depends on the Neck and Trunk Muscle Strength, Deformation of the Spine, and the Range of Motion in the Limb Joints International Journal of Environmental Research and Public Health spinal muscular atrophy motor function scoliosis range of motion physiotherapy rehabilitation |
author_facet |
Agnieszka Stępień Ewa Gajewska Witold Rekowski |
author_sort |
Agnieszka Stępień |
title |
Motor Function of Children with SMA1 and SMA2 Depends on the Neck and Trunk Muscle Strength, Deformation of the Spine, and the Range of Motion in the Limb Joints |
title_short |
Motor Function of Children with SMA1 and SMA2 Depends on the Neck and Trunk Muscle Strength, Deformation of the Spine, and the Range of Motion in the Limb Joints |
title_full |
Motor Function of Children with SMA1 and SMA2 Depends on the Neck and Trunk Muscle Strength, Deformation of the Spine, and the Range of Motion in the Limb Joints |
title_fullStr |
Motor Function of Children with SMA1 and SMA2 Depends on the Neck and Trunk Muscle Strength, Deformation of the Spine, and the Range of Motion in the Limb Joints |
title_full_unstemmed |
Motor Function of Children with SMA1 and SMA2 Depends on the Neck and Trunk Muscle Strength, Deformation of the Spine, and the Range of Motion in the Limb Joints |
title_sort |
motor function of children with sma1 and sma2 depends on the neck and trunk muscle strength, deformation of the spine, and the range of motion in the limb joints |
publisher |
MDPI AG |
series |
International Journal of Environmental Research and Public Health |
issn |
1661-7827 1660-4601 |
publishDate |
2021-08-01 |
description |
The purpose of this study was to investigate the functional relationships between selected ranges of motion of the neck, upper and lower limbs, the strength of the neck and trunk muscles, postural parameters, and the motor function of children with SMA1 and SMA2—27 children, aged 6 months-15 years, with genetically confirmed spinal muscular atrophy type 1 (19 children) and 2 (8 children) undergoing pharmacological treatment. All children were examined, according to the methodology, including the motor function evaluation, measurement of selected ranges of motion, assessment of postural parameters, and measurement of neck and trunk muscle strength. The functional status of 15 children was assessed with the CHOP INTEND (CHOP group) scale and of 12 children with the HFMSE (HFMSE group). The results obtained showed that, in children examined with the CHOP scale, greater limitation of flexion in the shoulder joints was observed. As the deformation of the chest increased, the functional abilities of children deteriorated. In participants examined with the CHOP group, the ranges of neck rotation decreased with the increase of the chest deformity. In the HFMSE group, the ranges of head rotation showed a strong relationship with some parameters of muscle strength and the sum of the R coefficients. Participants showed many significant relationships between the range of motion in the neck and joints of the limbs, with more significant relationships in the CHOP group. The following conclusions were made: motor skills of children with SMA depend on muscle strength, range of motion, and deformities of the spine and chest; the development of scoliosis adversely affects the motor function, ranges of motion, and muscle strength; and movement ranges are related to motor skills and strength values. |
topic |
spinal muscular atrophy motor function scoliosis range of motion physiotherapy rehabilitation |
url |
https://www.mdpi.com/1660-4601/18/17/9134 |
work_keys_str_mv |
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