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Neuromuscular disorders are conditions that impair the skeletal or voluntary muscle system. Strictly speaking, these afflictions are primary defects of muscle and the peripheral nervous system. Conditions resulting in impaired central control of muscles, however, are commonly considered secondary neuromuscular disorders with regard to treatment or research.
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The spectrum of primary neuromuscular and secondary nervous system disorders is expansive and can be found in Table 217-1. This chapter first focuses on basic orthopedic interventions, both operative and nonoperative, that are commonly applied in the course of management for neuromuscular disorders. This topic is followed by a review of selected neuromuscular disorders that require orthopedic attention and has been expanded to include arthrogryposis, as the general treatment principles are similar and not covered in other textbook sections. Where appropriate, the disease process and medical management are cross-referenced to chapters that provide greater details of specific entities.
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OVERVIEW OF MANAGEMENT
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NONOPERATIVE: ORTHOTICS AND BRACING
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The general purpose of orthoses is to help patients achieve stability in standing or walking and control the position of the extremities. They serve a purpose in both correction and prevention of deformities and may act as a compensatory mechanism for deficient muscle strength or activity. Orthoses may be considered positional (comparatively more rigid) or functional (adaptive to movement). The following paragraphs provide a brief introduction to common orthotic devices.
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Neuromuscular scoliosis can result from a variety of neuropathic or muscular conditions including cerebral palsy, myelodysplasia, and muscular dystrophy. Orthoses and bracing are commonly used in the early management of spinal disorders in pediatric patients, particularly to facilitate sitting, but generally are viewed as a temporary measure until the timing of surgical intervention is ideal. Unlike in idiopathic scoliosis, bracing does little to prevent progression of the curve. The most commonly used brace is the thoracolumbosacral orthosis (TLSO).