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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.

BackgroundThe majority of individuals with spinal muscular atrophy (SMA) experience progressive skeletal deformities which may affect the quality of life and mobility. To date, no studies have evaluated the reliability of tests assessing body posture and joint mobility in SMA patients. The purpose of this study was to assess the reliability of Cervical Rotation test (CR), Supine Angle of Trunk Rotation test (SATR), Hip Extension test (HE) and Pelvic Obliquity test (PO) developed to evaluate the musculoskeletal system in SMA individuals.MethodsThirty individuals (12 girls and 18 boys) aged 4–15 with SMA type II (n = 24) and III (n = 6) confirmed by genetic examinations were qualified for the study. The participants were examined twice by three physiotherapists on the same day. The examination included four tests, i.e. CR, SATR, HE and PO tests aimed at assessing ranges of rotation in the cervical spine, chest deformities, ranges of hip extension and pelvis position while sitting. Statistical calculations were made with the use of statistical software IBM SPSS Statistics version 20. Reliability was assessed using the Intraclass Correlation Coefficient (ICC).ResultsIntraobserver reliability was excellent for CR (ICC range 0.839–0.911), SATR (ICC range 0.918–0.939 - the upper part of the sternum; ICC range 0.951–0.975 - the lower part of the sternum), HE (ICC range 0.988–0,991) and PO (ICC range 0.896–0.935) tests.The interobserver ICC reached the excellent values in CR (ICC range 0.912–0.920), SATR (ICC = 0.888 - the upper part of the sternum, ICC = 0.951 - the lower part of the sternum), HE (ICC range 0.922–0.923) and PO (ICC = 0.928) tests.ConclusionsCR, SATR, HE and PO tests are reliable and may be used for examining individuals with SMA. The application of these tests provides a possibility to detect early changes in the musculoskeletal system in children and adolescents and to assess the effectiveness of the implemented pharmacotherapy and rehabilitation.

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IntroductionCentral nervous system damage in early life results in both quantitative and qualitative abnormalities of psychomotor development. Late sequelae of these disturbances may include visual perception disorders which not only affect the ability to read and write but also generally influence the child's intellectual development. This study sought to determine whether a central coordination disorder (CCD) in early life treated according to Vojta's method with elements of the sensory integration (S-I) and neuro-developmental treatment (NDT)/Bobath approaches affects development of visual perception later in life.Material and methodsThe study involved 44 participants aged 15-16 years, including 19 diagnosed with moderate or severe CCD in the neonatal period, i.e. during the first 2-3 months of life, with diagnosed mild degree neonatal encephalopathy due to perinatal anoxia, and 25 healthy people without a history of developmental psychomotor disturbances in the neonatal period. The study tool was a visual perception IQ test comprising 96 graphic tasks.ResultsThe study revealed equal proportions of participants (p < 0.05) defined as very skilled (94-96), skilled (91-94), aerage (71-91), poor (67-71), and very poor (0-67) in both groups. These results mean that adolescents with a history of CCD in the neonatal period did not differ with regard to the level of visual perception from their peers who had not demonstrated psychomotor development disorders in the neonatal period.ConclusionsEarly treatment of children with CCD affords a possibility of normalising their psychomotor development early enough to prevent consequences in the form of cognitive impairments in later life.

Background Musculoskeletal disorders are often observed in patients with spinal muscular atrophy (SMA). The aim of the study was to assess passive ranges of rotation in the cervical spine, chest deformity and pelvic obliquity in SMA patients, and to compare these results to the norms obtained in the group of healthy individuals. The second aim was to review these measurements and Cobb angle values for correlations in SMA patients. Methods The study included 74 patients with SMA and 89 healthy individuals aged 2 to 18 years. Cervical Rotation (CR), Supine Angle of Trunk Rotation (SATR) and Pelvic Obliquity (PO) tests were carried out. Results Cervical rotation ranges were significantly higher in the control group than in SMA patients (p < 0.05). Differences between cervical rotation ranges to the left and to the right were significantly larger in SMA I and SMA II groups than in healthy individuals (p = 0.000). Chest asymmetry and pelvic obliquity were bigger in SMA groups than in the control (p < 0.05). Significant correlations between cervical rotation measurements, chest deformity, pelvic obliquity and Cobb angle were found in SMA individuals, depending on the type. Conclusions The results of the study suggest that CR, SATR and PO tests may assist in the assessment of SMA patients in addition to the radiographic evaluation of the spine. Biomechanical relationships between disorders located in various skeletal structures should be taken into account in the treatment of SMA patients. Special attention should be given to assessing postural parameters in non- sitters and sitters. Treatment of patients with SMA and associated musculoskeletal disorders requires a multi-specialist approach.

Introduction: The Trunk-Pelvis-Hip Angle (TPHA) test is used for assessing the mobility of lumbo-pelvic-hip complex. The aim of the research was to assess the intraobserver reliability of the TPHA test in girls with and without idiopathic scoliosis and to compare the test values obtained by the girls in both groups. Material and methods: The research included girls without scoliosis and girls with double idiopathic scoliosis aged 8-16. The trunk-pelvis-hip angle was measured three times on each body side within one session. Statistical analysis was performed with the use of ANOVA intraclass correlation coefficient for dependent groups, Kolmogorov-Smirnov test as well as non-parametric Wilcoxon signed-rank test for dependent groups and Mann-Whitney U test for independent groups. Results: The research included 49 healthy girls (aged 11.8 ± SD 2.5 years) and 49 girls with double idiopathic scoliosis (aged 12.7 ± SD 2.6 years; Cobb angle Th 27.7° ± SD 13.4° and L Cobb 25.8° ± SD 10.5°). The intraobserver reliability for the TPHA measurements was very high (ICC>0.85). In both groups the left-side TPHA value was significantly higher than the right-side value (p=0.001). The TPHA range of motion on the right side of the body was more limited in the scoliotic girls than in the healthy ones (p=0.001). Conclusions: The TPHA test is a reliable means of assessing mobility in the lumbo-pelvic-hip complex in girls by one observer. The asymmetry of movements in the lumbo-pelvic-hip complex in healthy girls needs to be observed since it may constitute one of the factors predisposing to scoliosis which limits spine rotation range of motion. The TPHA test needs further research.

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