The 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. Thirty 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). Intraobserver 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. CR, 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.

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: Obesity is a common health issue among children and adolescents leading to serious consequences later in life. There are reports on postural stability in children in relation to their nutritional status, but there are few studies involving a stabilometric platform. Aim: A comparison of postural stability in children with normal body mass and obese children. Material and methods: The stabilometric study involved 166 subjects. The postural stability evaluation was performed with the use of the Biodex Balance System platform. The following four postural stability tests were carried out: (1) eyes open, stable platform (SI0); (2) eyes open, level 4 unstable platform (SI4); (3) eyes open, level 4 unstable platform with the screen covered (SI4SC); (4) eyes closed, level 4 unstable platform (SI4EC), and the fall risk test (FRT). Results and Discussion: There were statistically significant differences found in the SI4, SI4SC, SI4EC, and FRT tests in all the inspected parameters. Only in the postural stability test on a stable platform (SI0) no differences were noted. The most marked differences between arithmetic means obtained for both groups were observed in the postural stability test on an unstable platform with closed eyes. Conclusions: Children with obesity are characterised by increased instability on a moving surface compared to children of normal body mass. Obese children experience the biggest problem when regaining balance with their eyes closed. It is advisable to include training on an unstable surface in physiotherapy programmes for obese children.

Introduction: Recommendations for management of spinal muscular atrophy (SMA) do not contain detailed information about the position of lower limbs during support standing. It has been observed that during the measurement of the range of extension in the hip joint (HE) in SMA patients, the examined limb was often naturally abducted. Aim: The main aim of the study was to compare the values of HE in the sagittal plane and in abduction, and to assess the correlation between the duration of supported standing and HE in SMA patients. Material and methods: The study group consisted of 75 SMA individuals aged 2–22 years and control group consisted of 202 healthy participants. The measurements were performed with the Rippstein plurimeter and goniometer. Results and discussion: Range of HE in SMA patients was larger in abduction than in the sagittal plane. A correlation was noted between the duration of supported standing and HE. Conclusions: Supported standing with hip joint abduction should be used in SMA patients. The obtained results broaden the knowledge about the biomechanics of hip joints in SMA patients.

Background/Objectives: Duchenne muscular dystrophy (DMD) leads to postural abnormalities and increased lumbar lordosis, which may affect gait and spinal load. This study aimed to assess the reliability of sagittal spinal curvature measurements using the Rippstein plurimeter and to analyze spinal curvature in ambulant boys with DMD compared to healthy peers. Additionally, the study examined the effect of lower limb positioning in standing on sagittal spinal alignment in boys with DMD and investigated the relationship between hip adduction and extension range and spinal alignment. Methods: The study included 42 boys with DMD and 36 healthy peers aged 5–14 years. In boys with DMD, spinal curvature was measured using the Rippstein plurimeter in two positions: feet in alignment with hip joints axis and with feet together. In healthy participants, measurements were taken in the first position only. Hip adduction and extension ranges were also assessed in both groups. Results: Plurimeter measurements demonstrated high reliability. Boys with DMD showed significantly increased cervical retraction, greater sternal deviation from the vertical, and increased lumbar lordosis compared to healthy peers. Lower limb positioning (adduction) altered sagittal spinal alignment. Hip adduction and extension ranges were decreased in the DMD group and showed a correlation with spinal alignment. Conclusions: The Rippstein plurimeter provides reliable measurements and is useful for monitoring posture in boys with DMD. Reduced hip mobility and lower limb positioning influence lumbar lordosis and should be considered in physiotherapy planning for DMD.

Alternating hemiplegia of Childhood (AHC) is a rare disease manifested by transient episodes of hemiplegia and other neurological disorders. Delayed motor development has been reported in patients with AHC, but detailed features of the motor impairment have not been described so far. The aim of the study was to evaluate gross motor function between attacks in a group of Polish patients with AHC. The interictal gross motor function was assessed using the Gross Motor Function AHC scale, which consisted of 41 motor tasks. The study group consisted of 10 patients with AHC older than 2 years of age. The control group consisted of 30 age- and gender-matched subjects. The results achieved in each of the 41 tasks by the study subjects were compared to the results obtained with controls using the non-parametric Mann–Whitney U-test. In tasks 38–41, mean times were compared between the study subjects and controls. The study revealed gross motor function impairment in patients with AHC. The greatest differences compared to controls concerned such skills as standing on toes, walking on toes, walking on heels, as well as running and hopping on one leg and on alternate legs. Significant impairment of the motor function of the upper limbs was also found. The study confirmed motor function impairment between attacks in patients with AHC. The study findings may indicate the need to introduce individualised physiotherapy management of patients with AHC.