Neuromuscular Scoliosis

40 yo male, CP, spastic quadriplegic, progressive loss of sitting balance.

Neuromuscular scoliosis is an irregular spinal curvature due to abnormalities of the myoneural (muscle-nerve) pathways of the body. It is generally most severe in nonambulatory patients. Curve progression is much more frequent than idiopathic scoliosis, and may continue into adulthood. Bracing does not prevent progression of the spinal curvature.

Physical Exam Findings
Depending on the etiology, patients may or may not be able to walk. Certain disorders allow children to walk when they are young, but become wheelchair dependent during adolescence. In non-ambulators, trunk imbalance is common as the curves increase in size. Larger curves often force children to use their han

ds to improve sitting stability. Pelvic obliquity (tilt) is also common leading to sitting intolerance, and increased likelihood of skin breakdown (decubitus ulcers). Larger thoracic curves (80+^o) or hyperlordotic curves (backward bend) may contribute to pulmonary problems.

Frequency
Because of the many causes of neuromuscular scoliosis, the incidence is variable (Table 1). The likelihood and severity of the curves tends to increase with the degree of neuromuscular involvement.

Myelodysplasia Kyphoscoliosis

Diagnosis	Incidence of Scoliosis
Cerebral palsy (2-limbs involved) Myelodysplasia (lower lumbar) Spinal muscle atrophy Friedreich ataxia Cerebral palsy (4-limbs involved) Duchenne muscular dystrophy Myelodysplasia (thoracic level) Traumatic paralysis (<10 years) Table 1:	25% 60% 67% 80% 80% 90% 100% 100%

Nonoperative Management
Unlike idiopathic scoliosis, bracing is typically not effective for treating neuromuscular scoliosis. Smaller curves can be braced or treated with wheelchair modifications (in non-ambulators) in order to improve function.

It is important to have a multidisciplinary team evaluate and treat the patients as necessary. Pulmonary, neurologic, genitourinary, orthopaedic, nutritional and gastroenterologic issues are common and must be coordinated between health care providers.

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