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Published in: BMC Musculoskeletal Disorders 1/2019

Open Access 01-12-2019 | Spinal Muscular Atrophy | Research article

Reliability of four tests to assess body posture and the range of selected movements in individuals with spinal muscular atrophy

Authors: Agnieszka Stępień, Maria Jędrzejowska, Katarzyna Guzek, Witold Rekowski, Jolanta Stępowska

Published in: BMC Musculoskeletal Disorders | Issue 1/2019

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Abstract

Background

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.

Methods

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).

Results

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.

Conclusions

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.
Literature
1.
go back to reference Lefebvre S, Buirglen L, Reboullet S, Clermont O, Burlet P, Viollet L, et al. Identification and characterization of a spinal muscular atrophy-determining gene. Cell. 1995;80:155–65.CrossRef Lefebvre S, Buirglen L, Reboullet S, Clermont O, Burlet P, Viollet L, et al. Identification and characterization of a spinal muscular atrophy-determining gene. Cell. 1995;80:155–65.CrossRef
2.
go back to reference D’ Amico A, Mercuri E, D Tiziano F, Bertini E. Spinal muscular atrophy. Orphanet J Rare Dis. 2011;6:71.CrossRef D’ Amico A, Mercuri E, D Tiziano F, Bertini E. Spinal muscular atrophy. Orphanet J Rare Dis. 2011;6:71.CrossRef
3.
go back to reference Rodillo E, Marini ML, Heckmatt JZ, Dubowitz V. Scoliosis in spinal muscular atrophy: review of 63 cases. J Child Neurol. 1989;4:118–23.CrossRef Rodillo E, Marini ML, Heckmatt JZ, Dubowitz V. Scoliosis in spinal muscular atrophy: review of 63 cases. J Child Neurol. 1989;4:118–23.CrossRef
4.
go back to reference Carter GT, Abresch RT, Fowler WM, Johnson ER, Kilmer DD, McDonald CM. Profiles of neuromuscular diseases. Spinal muscular atrophy. Am J Phys Med Rehabil. 1995;74(5):150–9.CrossRef Carter GT, Abresch RT, Fowler WM, Johnson ER, Kilmer DD, McDonald CM. Profiles of neuromuscular diseases. Spinal muscular atrophy. Am J Phys Med Rehabil. 1995;74(5):150–9.CrossRef
5.
go back to reference Robinson D, Galasko CS, Delaney C, Williamson JB, Barrie JL. Scoliosis and lung function in spinal muscular atrophy. Eur Spine J. 1995;4:268–73.CrossRef Robinson D, Galasko CS, Delaney C, Williamson JB, Barrie JL. Scoliosis and lung function in spinal muscular atrophy. Eur Spine J. 1995;4:268–73.CrossRef
6.
go back to reference Chng SY, Wong YQ, Hui JH, Wong HK, Ong HT, Goh DY. Pulmonary function and scoliosis in children with spinal muscular atrophy types II and III. J Paediatr Child Health. 2003;39:673–6.CrossRef Chng SY, Wong YQ, Hui JH, Wong HK, Ong HT, Goh DY. Pulmonary function and scoliosis in children with spinal muscular atrophy types II and III. J Paediatr Child Health. 2003;39:673–6.CrossRef
7.
go back to reference Wang CH, Finkel RS, Bertini ES, Schroth M, Simonds A, Wong B, et al. Consensus statement for standard of Care in Spinal Muscular Atrophy. J Child Neurol. 2007;22:1027–249.CrossRef Wang CH, Finkel RS, Bertini ES, Schroth M, Simonds A, Wong B, et al. Consensus statement for standard of Care in Spinal Muscular Atrophy. J Child Neurol. 2007;22:1027–249.CrossRef
8.
go back to reference Mullender MG, Blom NA, De Kleuver M, Fock JM, Hitters WM, Horemans AM, et al. A Dutch guideline for the treatment of scoliosis in neuromuscular disorders. Scoliosis. 2008;3:14.CrossRef Mullender MG, Blom NA, De Kleuver M, Fock JM, Hitters WM, Horemans AM, et al. A Dutch guideline for the treatment of scoliosis in neuromuscular disorders. Scoliosis. 2008;3:14.CrossRef
9.
go back to reference Canavese F, Sussman MD. Strategies of hip management in neuromuscular disorders: Duchenne muscular dystrophy, spinal muscular atrophy, Charcot-Marie-tooth disease and arthrogryposis multiplex Congenita. Hip Int. 2009;19(6):46–52.CrossRef Canavese F, Sussman MD. Strategies of hip management in neuromuscular disorders: Duchenne muscular dystrophy, spinal muscular atrophy, Charcot-Marie-tooth disease and arthrogryposis multiplex Congenita. Hip Int. 2009;19(6):46–52.CrossRef
10.
go back to reference Fujak A, Kopschina C, Gras F, Forst R, Forst J. Contractures of the lower extremities in spinal muscular atrophy type II. Descriptive clinical study with retrospective data collection. Ortop Traumatol Rehabil. 2011;13:27–36.CrossRef Fujak A, Kopschina C, Gras F, Forst R, Forst J. Contractures of the lower extremities in spinal muscular atrophy type II. Descriptive clinical study with retrospective data collection. Ortop Traumatol Rehabil. 2011;13:27–36.CrossRef
11.
go back to reference Hull J, Aniapravan R, Chan E, Chatwin M, Forton J, Gallagher J, et al. British Thoracic Society guideline for respiratory management of children with neuromuscular weakness. Thorax. 2012;67(1):1–40.CrossRef Hull J, Aniapravan R, Chan E, Chatwin M, Forton J, Gallagher J, et al. British Thoracic Society guideline for respiratory management of children with neuromuscular weakness. Thorax. 2012;67(1):1–40.CrossRef
12.
go back to reference Fujak A, Raab W, Schuh A, Richter S, Forst R, Forst J. Natural course of scoliosis in proximal spinal muscular atrophy type II and IIIa: descriptive clinical study with retrospective data collection of 126 patients. BMC Musculoskelet Disord. 2013;14:283.CrossRef Fujak A, Raab W, Schuh A, Richter S, Forst R, Forst J. Natural course of scoliosis in proximal spinal muscular atrophy type II and IIIa: descriptive clinical study with retrospective data collection of 126 patients. BMC Musculoskelet Disord. 2013;14:283.CrossRef
13.
go back to reference Mercuri E, Finkel RS, Muntoni F, Wirth B, Montes J, Main M, et al. Diagnosis and management of spinal muscular atrophy: part 1: recommendations for diagnosis, rehabilitation orthopedic and nutritional care. Neuromuscul Disord. 2018;28(2):103–15.CrossRef Mercuri E, Finkel RS, Muntoni F, Wirth B, Montes J, Main M, et al. Diagnosis and management of spinal muscular atrophy: part 1: recommendations for diagnosis, rehabilitation orthopedic and nutritional care. Neuromuscul Disord. 2018;28(2):103–15.CrossRef
14.
go back to reference Iannaccone ST, Hynan LS. American spinal muscular atrophy randomized trials (AmSMART) group. Reliability of 4 outcome measures in pediatric spinal muscular atrophy. Arch Neurol. 2003;60(8):1130–6.CrossRef Iannaccone ST, Hynan LS. American spinal muscular atrophy randomized trials (AmSMART) group. Reliability of 4 outcome measures in pediatric spinal muscular atrophy. Arch Neurol. 2003;60(8):1130–6.CrossRef
15.
go back to reference Main M, Kairon H, Mercuri E, Muntoni F. The Hammersmith functional motor scale for children with spinal muscular atrophy: a scale to test ability and monitor progress in children with limited ambulation. Eur J Paediatr Neurol. 2003;7:155–9.CrossRef Main M, Kairon H, Mercuri E, Muntoni F. The Hammersmith functional motor scale for children with spinal muscular atrophy: a scale to test ability and monitor progress in children with limited ambulation. Eur J Paediatr Neurol. 2003;7:155–9.CrossRef
16.
go back to reference Barois A, Mayer M, Desguerre I, Chabrol B, Berard C, Cuisset JM, et al. Spinal muscular atrophy. A 4-year prospective, multicenter, longitudinal study (168 cases). Bull Acad Natl Med. 2005;189:1181–99.PubMed Barois A, Mayer M, Desguerre I, Chabrol B, Berard C, Cuisset JM, et al. Spinal muscular atrophy. A 4-year prospective, multicenter, longitudinal study (168 cases). Bull Acad Natl Med. 2005;189:1181–99.PubMed
17.
go back to reference Nelson L, Owens H, Hynan LS, Iannaccone ST, AmSMART Group. The gross motor function measure is a valid and sensitive outcome measure for spinal muscular atrophy. Neuromuscul Disord. 2006;16:374–80.CrossRef Nelson L, Owens H, Hynan LS, Iannaccone ST, AmSMART Group. The gross motor function measure is a valid and sensitive outcome measure for spinal muscular atrophy. Neuromuscul Disord. 2006;16:374–80.CrossRef
18.
go back to reference Mercuri E, Messina S, Battini R, Berardinelli A, Boffi P, Bono R, et al. Reliability of the Hammersmith functional motor scale for spinal muscular atrophy in a multicentric study. Neuromuscul Disord. 2006;16:93–8.CrossRef Mercuri E, Messina S, Battini R, Berardinelli A, Boffi P, Bono R, et al. Reliability of the Hammersmith functional motor scale for spinal muscular atrophy in a multicentric study. Neuromuscul Disord. 2006;16:93–8.CrossRef
19.
go back to reference Krosschell KJ, Maczulski JA, Crawford TO, Scott C, Swoboda KJ. A modified Hammersmith functional motor scale for use in multi-center research on spinal muscular atrophy. Neuromuscul Disord. 2006;16:417–26.CrossRef Krosschell KJ, Maczulski JA, Crawford TO, Scott C, Swoboda KJ. A modified Hammersmith functional motor scale for use in multi-center research on spinal muscular atrophy. Neuromuscul Disord. 2006;16:417–26.CrossRef
20.
go back to reference Finkel RS, Glanzman AM, Main M, Bertini E, Mercuri E. The CHOP INTEND: a reliable motor scale for infants with neuromuscular disease. Neuromuscul Disord. 2006;16:9–10.CrossRef Finkel RS, Glanzman AM, Main M, Bertini E, Mercuri E. The CHOP INTEND: a reliable motor scale for infants with neuromuscular disease. Neuromuscul Disord. 2006;16:9–10.CrossRef
21.
go back to reference Dunaway YS, Montes J, Kramer SS, Marra J, Salazar R, Cruz R, et al. Six-minute walk test is reliable and valid in spinal muscular atrophy. Muscle Nerve. 2016;54(5):836–84.CrossRef Dunaway YS, Montes J, Kramer SS, Marra J, Salazar R, Cruz R, et al. Six-minute walk test is reliable and valid in spinal muscular atrophy. Muscle Nerve. 2016;54(5):836–84.CrossRef
22.
go back to reference Merlini L, Mazzone ES, Solari A, Morandi L. Reliability of hand-held dynamometry in spinal muscular atrophy. Muscle Nerve. 2002;26:64–70.CrossRef Merlini L, Mazzone ES, Solari A, Morandi L. Reliability of hand-held dynamometry in spinal muscular atrophy. Muscle Nerve. 2002;26:64–70.CrossRef
23.
go back to reference Wang HY, Ju YH, Chen SM, Lo SK, Jong YJ. Joint range of motion limitations in children and young adults with spinal muscular atrophy. Arch Phys Med Rehabil. 2004;85:1689–93.CrossRef Wang HY, Ju YH, Chen SM, Lo SK, Jong YJ. Joint range of motion limitations in children and young adults with spinal muscular atrophy. Arch Phys Med Rehabil. 2004;85:1689–93.CrossRef
24.
go back to reference Sporer SM, Smith BG. Hip dislocation in patients with spinal muscular atrophy. J Pediatr Orthop. 2003;23:10–4.PubMed Sporer SM, Smith BG. Hip dislocation in patients with spinal muscular atrophy. J Pediatr Orthop. 2003;23:10–4.PubMed
25.
go back to reference Birnkrant DJ, Bushby K, Bann CM, Alman BA, Apkon SD, Blackwell A, Case LE, et al. Diagnosis and management of Duchenne muscular dystrophy, part 2: respiratory, cardiac, bone health, and orthopaedic management. Lancet Neurol. 2018;17(4):347–61.CrossRef Birnkrant DJ, Bushby K, Bann CM, Alman BA, Apkon SD, Blackwell A, Case LE, et al. Diagnosis and management of Duchenne muscular dystrophy, part 2: respiratory, cardiac, bone health, and orthopaedic management. Lancet Neurol. 2018;17(4):347–61.CrossRef
26.
go back to reference Grivas TB, Vasiliadis ES, Koufopoulos G, Segos D, Triantafyllopoulos G, Mouzakis V. Study of trunk asymmetry in normal children and adolescents. Scoliosis. 2006;1:19.CrossRef Grivas TB, Vasiliadis ES, Koufopoulos G, Segos D, Triantafyllopoulos G, Mouzakis V. Study of trunk asymmetry in normal children and adolescents. Scoliosis. 2006;1:19.CrossRef
27.
go back to reference Negrini S, Aulisa AG, Aulisa L, Circo AB, de Mauroy JC, Durmala J, et al. 2011 SOSORT guidelines: Orthopaedic and rehabilitation treatment of idiopathic scoliosis during growth. Scoliosis. 2012;7:3.CrossRef Negrini S, Aulisa AG, Aulisa L, Circo AB, de Mauroy JC, Durmala J, et al. 2011 SOSORT guidelines: Orthopaedic and rehabilitation treatment of idiopathic scoliosis during growth. Scoliosis. 2012;7:3.CrossRef
28.
go back to reference Amendt LE, Ause-Ellias KL, Eybers JL, Wadsworth CT, Nielsen DH, Weinstein SL. Validity and reliability testing of the Scoliometer. Phys Ther. 1990;70(Suppl):108–17.CrossRef Amendt LE, Ause-Ellias KL, Eybers JL, Wadsworth CT, Nielsen DH, Weinstein SL. Validity and reliability testing of the Scoliometer. Phys Ther. 1990;70(Suppl):108–17.CrossRef
29.
go back to reference Korovessis PG. Scoliometer is useful instrument with high reliability and repeatability. Spine (Phila Pa 1976). 1999;24(3):307–8.CrossRef Korovessis PG. Scoliometer is useful instrument with high reliability and repeatability. Spine (Phila Pa 1976). 1999;24(3):307–8.CrossRef
30.
go back to reference Bonagamba GH, Coelho DM, Oliveira A. Inter and intra-rater reliability of the scoliometer. Rev Bras Fisioter. 2010;14(5):432–8.CrossRef Bonagamba GH, Coelho DM, Oliveira A. Inter and intra-rater reliability of the scoliometer. Rev Bras Fisioter. 2010;14(5):432–8.CrossRef
31.
go back to reference Green S, Buchbinder R, Forbes A, Bellamy N. A standardized protocol for measurement of range of movement of the shoulder using the Plurimeter-V inclinometer and assessment of its intrarater and interrater reliability. Arthritis Care Res. 1998;11(1):43–52.CrossRef Green S, Buchbinder R, Forbes A, Bellamy N. A standardized protocol for measurement of range of movement of the shoulder using the Plurimeter-V inclinometer and assessment of its intrarater and interrater reliability. Arthritis Care Res. 1998;11(1):43–52.CrossRef
32.
go back to reference Watson L, Balster SM, Finch C, Dalziel R. Measurement of scapula upward rotation: a reliable clinical procedure. Br J Sports Med. 2005;39:599–603.CrossRef Watson L, Balster SM, Finch C, Dalziel R. Measurement of scapula upward rotation: a reliable clinical procedure. Br J Sports Med. 2005;39:599–603.CrossRef
33.
go back to reference De Groef A, Van Kampen M, Vervloesem N, Clabau E, Christiaens MR, Neven P, et al. Inter-rater reliability of shoulder measurements in middle-aged women. Physiotherapy. 2016;103(2):222–30.CrossRef De Groef A, Van Kampen M, Vervloesem N, Clabau E, Christiaens MR, Neven P, et al. Inter-rater reliability of shoulder measurements in middle-aged women. Physiotherapy. 2016;103(2):222–30.CrossRef
34.
go back to reference Norkin CC, White DJ. Measurement of joint motion: a guide to goniometry. 3th ed. Philadelphia: FA Davis; 2003. Norkin CC, White DJ. Measurement of joint motion: a guide to goniometry. 3th ed. Philadelphia: FA Davis; 2003.
35.
go back to reference Shrout PE, Fleiss JL. Intraclass correlations: uses in assessing rater reliability. Psychol Bull. 1979;86:420–8.CrossRef Shrout PE, Fleiss JL. Intraclass correlations: uses in assessing rater reliability. Psychol Bull. 1979;86:420–8.CrossRef
36.
go back to reference Cicchetti DV. Guidelines, criteria, and rules of thumb for evaluating normed and standardized assessment instruments in psychology. Psychol Assess. 1994;6(4):284–90.CrossRef Cicchetti DV. Guidelines, criteria, and rules of thumb for evaluating normed and standardized assessment instruments in psychology. Psychol Assess. 1994;6(4):284–90.CrossRef
37.
go back to reference van den Engel-Hoek L, de Swart BJ, Erasmus CE, de Groot IJ. Is head balance a major determinant for swallowing problems in patients with spinal muscular atrophy type 2? J Child Neurol. 2008;23(8):919–21.CrossRef van den Engel-Hoek L, de Swart BJ, Erasmus CE, de Groot IJ. Is head balance a major determinant for swallowing problems in patients with spinal muscular atrophy type 2? J Child Neurol. 2008;23(8):919–21.CrossRef
38.
go back to reference Van Dillen LR, McDonnell MK, Fleming DA, Sahrmann SA. Effect of knee and hip position on hip extension range of motion in individuals with and without low back pain. J Orthop Sports Phys Ther. 2000;30:307.CrossRef Van Dillen LR, McDonnell MK, Fleming DA, Sahrmann SA. Effect of knee and hip position on hip extension range of motion in individuals with and without low back pain. J Orthop Sports Phys Ther. 2000;30:307.CrossRef
39.
go back to reference Pandya S, Florence JM, King WM, Robison JD, Oxman M, Province MA. Reliability of goniometric measurements in patients with Duchenne muscular dystrophy. Phys Ther. 1985;65(9):1339–42.CrossRef Pandya S, Florence JM, King WM, Robison JD, Oxman M, Province MA. Reliability of goniometric measurements in patients with Duchenne muscular dystrophy. Phys Ther. 1985;65(9):1339–42.CrossRef
Metadata
Title
Reliability of four tests to assess body posture and the range of selected movements in individuals with spinal muscular atrophy
Authors
Agnieszka Stępień
Maria Jędrzejowska
Katarzyna Guzek
Witold Rekowski
Jolanta Stępowska
Publication date
01-12-2019
Publisher
BioMed Central
Published in
BMC Musculoskeletal Disorders / Issue 1/2019
Electronic ISSN: 1471-2474
DOI
https://doi.org/10.1186/s12891-018-2389-8

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