Neuromuscular Scoliosis
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Introduction[edit | edit source]
Neuromuscular scoliosis (NMS) is defined as a non-congenital spinal deformity present in conjunction with any type of pre-existing neuromuscular diagnosis.[1]NMS is generally more severe than adolescent idiopathic scoliosis (AIS) and more progressive, typically continuing after the growth of the individual and spine has ceased.[2]Its progressive nature is more often seen in conditions of neurologic and systemic involvement[1].
Etiology[edit | edit source]
According to the Scoliosis Research Society spinal deviation can be: NMS the basics
- Neuropathic with central or peripheral motor neuron involvement (or both)
- Myopathic
Neuromuscular scoliosis is a common consequence of neuromotor disease. Examples of these include[2]:
- Cerebral Palsy
- Spinal cord injury
- Motor neuron disease, e.g. Spinal muscular atrophy
- Muscle fibre disorders, e.g. Duchenne muscular atrophy
- Multifocal disorders, e.g. Spina Bifida
In diseases of motor neuron and muscle fibre disease, onset of scoliosis usually occurs once the individual loses ambulation. Age and onset have not been found to be a predictor of progression and severity of the deformity.[3]
Pathogenesis[edit | edit source]
NMS is not simply a result of trunk muscle weakness. Instead, hypertonia is more often found to be the result of hypertonia.
These conditions and their own etiology vary widely, however, there are often common factors which contribute to the spinal deformity. These include[2]:
- Asymmetrical paraplegia
- imbalance of mechanical forces
- Intraspinal and congenital abnormalities of the spine
- Altered sensory feedback
- Abnormal posture via central pathways
Orthopaedic manifestations are also often similar amongst different conditions[1].
Impairments[edit | edit source]
The result of a NMS is often limitations or impairments to the individual involved.
These include[2]:
- Pain
- Compromised cardiopulmonary function (secondary to mechanical effects on thoracic volume and compliance.
- Alterations in skin integrity
- Limited or decreased....
- Limb movement
- Gait
- Standing
- Sitting
- General balance capacity
- Trunk stabilty
- Bimanual activities
- Functioning in activities of Daily Living
- Complications in self image and social interactions
General management[edit | edit source]
Management includes [1]:
- Observation
- Conservative treatment
- Physiotherapy
- Bracing
In a 2019 study by Murphy and Mooney, it was highlighted that there remains a lack of high level evidence that supports the positive effect of conservative treatment of NMS. The evidence is most supportive of improved sitting balance in patients with flexible curves. In general, as deformity progresses, the effectiveness of bracing decreases. The study noted some improvements in Cobb angles and sitting stability with a 3-point molded brace.
- Seating systems
- Surgery
- Treatment of the underlying pathology.
For example steroid administration to those diagnosed with DMD.
In dealing with NMS the goals of management include[4]:
- Preservation of function
- Facilitation of daily care
- Alleviation of pain
Management is best performed within a multidisciplinary team. While conservative management comprises of physiotherapy and bracing, surgery often plays an essential role in the rehabilitative process.[5]
Surgical management[edit | edit source]
Surgical intervention is usually prescribed when the measured Cobb's angle exceeds 40° (usually lying between 40-50°) but may be performed earlier in those diagnosed with conditions such as DMD.[4]
Indications for surgery are that of progressive deformity with an unacceptable truncal shift or pelvic obliquity that affects standing or sitting balance and/or positioning.[1]
The goal of surgical intervention is to obtain a solid bony arthrodesis of the spine and pelvis in order to restore seated or standing balance.[1]
Intraoperative and postoperative complications tend to be quite high. In a study by Weissmann et al (2021) complication rates were between 33.1% and 40.1% with similar percentages being reported in other reviews. Despite this, the same study found a significant decrease in urinary tract infections, lower respiratory tract infections and even epilepsy after surgery.
An important component to reducing these complications is to do a thorough patient history (including complications/ conditions present before birth). This would be done to pick up possible co-morbidities. NMS: comorbidities and complications
Physiotherapy management[edit | edit source]
Components to address include
- Respiratory function: In this aspect it is important to address the respiration
- Pain: A randomized control trial (RCT) published in 2017 showed that core stabilisation exercises were effective in decreasing pain and also decreasing rotational deformity. https://journals.lww.com/poijournal/Abstract/2017/41030/The_effectiveness_of_core_stabilization_exercise.11.aspx
Resources[edit | edit source]
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References[edit | edit source]
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 Murphy RF, Mooney JF. Current concepts in neuromuscular scoliosis. Current Reviews in Musculoskeletal Medicine. 2019 Jun;12(2):220-7.[1]
- ↑ 2.0 2.1 2.2 2.3 Allam AM, Schwabe AL. Neuromuscular scoliosis. PM&R. 2013 Nov 1;5(11):957-63.
- ↑ Mullender MG, Blom NA, De Kleuver M, Fock JM, Hitters WM, Horemans AM, Kalkman CJ, Pruijs JE, Timmer RR, Titarsolej PJ, Van Haasteren NC. A Dutch guideline for the treatment of scoliosis in neuromuscular disorders. Scoliosis. 2008 Dec;3(1):1-4.[2]
- ↑ 4.0 4.1 Roberts SB, Tsirikos AI. Factors influencing the evaluation and management of neuromuscular scoliosis: a review of the literature. Journal of back and musculoskeletal rehabilitation. 2016 Jan 1;29(4):613-23.[3]
- ↑ Kotwicki T, Jozwiak M. Conservative management of neuromuscular scoliosis: personal experience and review of literature. Disability and rehabilitation. 2008 Jan 1;30(10):792-8.[4]
