Prospective Analysis of Functional and Structural Changes in Patients with Spinal Muscular Atrophy—A Pilot Study
Spinal muscular atrophy (SMA) is a rare, autosomal recessive neuromuscular disease. Recent years have seen a significant development of therapeutic options for SMA patients. With the development of treatment methods, it has become necessary to adapt a physiotherapeutic approach to the evolving clini...
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| Format: | Article |
| Language: | English |
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MDPI AG
2022-12-01
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| Series: | Biomedicines |
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| Online Access: | https://www.mdpi.com/2227-9059/10/12/3187 |
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| author | Aleksandra Bieniaszewska Magdalena Sobieska Ewa Gajewska |
| author_facet | Aleksandra Bieniaszewska Magdalena Sobieska Ewa Gajewska |
| author_sort | Aleksandra Bieniaszewska |
| collection | DOAJ |
| description | Spinal muscular atrophy (SMA) is a rare, autosomal recessive neuromuscular disease. Recent years have seen a significant development of therapeutic options for SMA patients. With the development of treatment methods, it has become necessary to adapt a physiotherapeutic approach to the evolving clinical picture of SMA patients. We presented an analysis of 40 SMA patients undergoing pharmacological treatment, examined twice in an average interval of 5 months. Twelve patients (non-sitters) were evaluated using CHOP-INTEND, while 28 (sitters) were tested using the Hammersmith scale. The research protocol consisted of measurements of upper and lower limb ranges of motion, and four tests for early detection of musculoskeletal changes. Both non-sitters and sitters patients showed motor improvement between the first and second examinations. Favorable changes in range of motion parameters were noted in most children, except for hip extension (HE) range, which deteriorated. An association was also observed between scale scores and the presence of contractures in the hip and knee joints depending on the group studied. Our findings showed that the presence of contractures at the hip and knee joint negatively affected functional improvement as measured by the scale scores. |
| first_indexed | 2024-03-09T17:18:27Z |
| format | Article |
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| institution | Directory Open Access Journal |
| issn | 2227-9059 |
| language | English |
| last_indexed | 2024-03-09T17:18:27Z |
| publishDate | 2022-12-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Biomedicines |
| spelling | doaj.art-9eebe8bbc370423996779ac7d77fa3ed2023-11-24T13:28:22ZengMDPI AGBiomedicines2227-90592022-12-011012318710.3390/biomedicines10123187Prospective Analysis of Functional and Structural Changes in Patients with Spinal Muscular Atrophy—A Pilot StudyAleksandra Bieniaszewska0Magdalena Sobieska1Ewa Gajewska2Department of Developmental Neurology, Poznan University of Medical Sciences, 60-355 Poznan, PolandDepartment of Rehabilitation and Physiotherapy, Poznan University of Medical Sciences, 61-545 Poznan, PolandDepartment of Developmental Neurology, Poznan University of Medical Sciences, 60-355 Poznan, PolandSpinal muscular atrophy (SMA) is a rare, autosomal recessive neuromuscular disease. Recent years have seen a significant development of therapeutic options for SMA patients. With the development of treatment methods, it has become necessary to adapt a physiotherapeutic approach to the evolving clinical picture of SMA patients. We presented an analysis of 40 SMA patients undergoing pharmacological treatment, examined twice in an average interval of 5 months. Twelve patients (non-sitters) were evaluated using CHOP-INTEND, while 28 (sitters) were tested using the Hammersmith scale. The research protocol consisted of measurements of upper and lower limb ranges of motion, and four tests for early detection of musculoskeletal changes. Both non-sitters and sitters patients showed motor improvement between the first and second examinations. Favorable changes in range of motion parameters were noted in most children, except for hip extension (HE) range, which deteriorated. An association was also observed between scale scores and the presence of contractures in the hip and knee joints depending on the group studied. Our findings showed that the presence of contractures at the hip and knee joint negatively affected functional improvement as measured by the scale scores.https://www.mdpi.com/2227-9059/10/12/3187spinal muscular atrophyfunctional assessmentfunctional scalesranges of motioncontractures |
| spellingShingle | Aleksandra Bieniaszewska Magdalena Sobieska Ewa Gajewska Prospective Analysis of Functional and Structural Changes in Patients with Spinal Muscular Atrophy—A Pilot Study Biomedicines spinal muscular atrophy functional assessment functional scales ranges of motion contractures |
| title | Prospective Analysis of Functional and Structural Changes in Patients with Spinal Muscular Atrophy—A Pilot Study |
| title_full | Prospective Analysis of Functional and Structural Changes in Patients with Spinal Muscular Atrophy—A Pilot Study |
| title_fullStr | Prospective Analysis of Functional and Structural Changes in Patients with Spinal Muscular Atrophy—A Pilot Study |
| title_full_unstemmed | Prospective Analysis of Functional and Structural Changes in Patients with Spinal Muscular Atrophy—A Pilot Study |
| title_short | Prospective Analysis of Functional and Structural Changes in Patients with Spinal Muscular Atrophy—A Pilot Study |
| title_sort | prospective analysis of functional and structural changes in patients with spinal muscular atrophy a pilot study |
| topic | spinal muscular atrophy functional assessment functional scales ranges of motion contractures |
| url | https://www.mdpi.com/2227-9059/10/12/3187 |
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