Spinal curvature is a common concern, as it develops in more than 50% of individuals with spinal muscular atrophy, most commonly in those who are nonambulatory. This may be addressed by surgical correction or positional support (eg, bracing). The decision to perform surgical correction of complications such as scoliosis is based on a variety of factors, including degree of spine curvature, rate of progression, pulmonary function, and bone maturity.1,2
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SURGERY |
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BRACING |
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Limb contractures are common in neuromuscular diseases such as spinal muscular atrophy5
Contractures may occur in patients who have lost, or never achieved, the ability to ambulate. They contribute to reduced functional range of motion, loss of function for activities of daily living, and increased pain. One study found reductions in range of motion by >20° among 22% to 50% of patients with later-onset (consistent with Type II) spinal muscular atrophy. Hip, knee, and wrist contractures were most common.5 Orthopedic treatment often consists of range-of-motion exercises in order to prevent fixed joint contractures.6
Mobility-assistive technology
Power-assistive mobility devices may be considered for individuals with severe or progressive neuromuscular disease who suffer from fatigue and lack of endurance, allowing them to conserve energy and focus more on activities of daily living. Children with spinal muscular atrophy who are as young as 20 months of age have demonstrated safe, independent use of powered wheelchairs.7,8
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STANDING WHEELCHAIR9 |
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CONTROLLERS |
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References
1. Spinal Muscular Atrophy Clinical Research Center. Physical/occupational therapy. http://columbiasma.org/pt-ot.html. Updated March 14, 2013. Accessed April 18, 2016. 2. Mercuri E, Finkel RS, Muntoni F, et al; SMA Care Group. Diagnosis and management of spinal muscular atrophy: part 1: recommendations for diagnosis, rehabilitation, orthopedic and nutritional care. Neuromuscul Disord. 2018;28(2):103-115. 3. Mullender M, Blom N, De Kleuver M, et al. A Dutch guideline for the treatment of scoliosis in neuromuscular disorders. Scoliosis. 2008;3:14. 4. Tangsrud SE, Carlsen KC, Lund-Petersen I, Carlsen KH. Lung function measurements in young children with spinal muscular atrophy; a cross sectional survey on the effect of position and bracing. Arch Dis Child. 2001;84(6):521-524. 5. Skalsky AJ, McDonald CM. Prevention and management of limb contractures in neuromuscular diseases. Phys Med Rehabil Clin N Am. 2012;23(3):675-687. 6. Tsirikos AI, Baker ADL. Spinal muscular atrophy: classification, aetiology, and treatment of spinal deformity in children and adolescents. Curr Orthoped. 2006;20:430-445. 7. Lin W, Pierce A, Skalsky AJ, McDonald CM. Mobility-assistive technology in progressive neuromuscular disease. Phys Med Rehabil Clin N Am. 2012;23(4):885-894. 8. Jones MA, McEwen IR, Hansen L. Use of power mobility for a young child with spinal muscular atrophy. Phys Ther. 2003;83(3):253-262. 9. Arva J, Paleg G, Lange M, et al. RESNA position on the application of wheelchair standing devices. Assist Technol. 2009;21(3):161-168. 10. Hilliard K. Special Effect’s controllers enable every gamer to enjoy video games. http://www.gameinformer.com/b/features/archive/2014/06/25/creating-controllers-for-all-disabilities-special-effect-enables-every-gamer-to-enjoy-video-games.aspx. Game Informer website. Published June 25, 2014. Accessed August 1, 2016.

The clinical spectrum of SMA is highly variable and often requires comprehensive medical care involving a multidisciplinary approach.2