The development of spinal deformities is a significant problem in the disease course of neuromuscular disorders and requires a comprehensive and multidisciplinary approach. Neuromuscular scoliosis behaves somewhat differently from the idiopathic forms of scoliosis. Each disease has its own characteristics and even within the same neuromuscular disorder the curve can behave differently depending on the age of onset and the age of the individual. The typical curve pattern in neuromuscular disease is a long C-shaped collapsing kyphoscoliosis with pelvic obliquity which means the pelvis is not in a perfect horizontal position. However, an S-shaped type is often observed in some patients such as Duchenne muscular dystrophy and Charcot Marie Tooth.
The management of scoliosis in NMD requires a multidisciplinary approach and may include medication, bracing, spinal fusion and growing rods. The goal of any management of scoliosis is to prevent progression and restore sitting balance and the use of the upper limbs to improve quality of life. For example, a spinal jacket will not necessarily delay the progression of a scoliosis in a disease such as spinal muscular atrophy but may give sufficient trunk support to allow the individual to use their arms for functional activities of daily living rather than propping themselves in a sitting position.
More information on the management of paediatric neuromuscular scoliosis can be found in the book “Neuromuscular disorders in children” in Clinics in Developmental Medicine, Mac Keith Press. Edited by Nicholas Deconnick and Natalie Goemans.
Posture is something that physiotherapists refer to quite a lot and posture can be influenced by pain, muscle weakness and muscle activity. Posture refers to the way you hold yourself up against gravity and the position you adopt when you’re standing or sitting or lying down. Posture is not just about static positions but also dynamic activities such as walking running and moving in a wheelchair. In many neuromuscular disorders muscle weakness impacts posture.
Resources on Postural Management
This leaflet from the Scottish Muscle Network offers advice around postural management in standing, sitting, in wheelchairs and when lying and is a useful leaflet to pass on to patients. Information on postural management