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Neuromuscular Scoliosis

Neuromuscular disorders commonly lead to the spinal deformities of scoliosis and kyphosis, which have similar features despite the varied pathology of the associated conditions.

The etiology of neuromuscular spinal deformity can be categorized based on the underlying disorder. The primary abnormality may either be the nervous system or the muscles, and both may lead to truncal imbalance with spinal deformity. Neuromuscular disorders associated with scoliosis and/or kyphosis have been classified by the Scoliosis Research Society. Neuropathic conditions may have either upper motor neuron (e.g., cerebral palsy [CP]) or lower motor neuron (e.g., poliomyelitis) pathology, Myopathic conditions (e.g., arthrogryposis, muscular dystrophy) are also commonly associated with the development of a spinal deformity.

The scoliosis associated with these neuromuscular disorders is generally known for early onset with rapid progression during growth, as well as continued progression after growth is complete. This may lead to further compromise of functional abilities. With increasing deformity, these patients develop limited sitting tolerance, back pain, and compromised pulmonary function.

Associated with these curves is also the failure of the pelvis to remain horizontal to the sitting surface and perpendicular to the spine. This pelvic obliquity contributes to uneven weight bearing in the sitting position that causes increased skin pressure and pain in patients with normal sensation or risk of pressure sores for those patients without protective sensation.1 An oblique pelvis can also compromise functional abilities by necessitating the use of the patient's hands/upper extremities to support the trunk, rather than leaving the hands free for more useful activities.

Example

The principles and goals of treatment are similar for all types of spinal deformity. In neuromuscular disorders, and in neurologic muscular scoliosis in particular, the natural history of the underlying disease is an important determinant of which patient to treat and with which method. The treatment goal in general focuses around limiting curve progression and maintaining maximal function. The basic tenets of observing mild curves, bracing moderate curves, and fusing severe curves apply to neuromuscular scoliosis, although the particulars of a specific disorder or patient will often dictate the treatment method

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