A rare cause of neuromuscular scoliosis: Alexander disease

Alexander disease belongs to a group of progressive neurological disorders in which the destruction of white matter in the brain is accompanied by the formation of fibrous, eosinophilic deposits known as Rosenthal fibers. Spinal deformity rarely accompanies this disease. The aim of this report is to present a patient with juvenile-onset Alexander disease, who also had progressive neuromuscular type scoliosis requiring surgical stabilization. A 13-year-old male presented with bilateral weakness in both lower extremities and scoliosis. Results of an examination of the spine showed a left thoracic scoliosis with prominent left paraspinous prominence and elevation of the ipsilateral shoulder. Spinal fusion with rigid internal stabilizing instrumentation was selected for surgical treatment of the scoliosis. The fusion area was to be from T2 to L4. He was instrumented with pedicle screw fixation system, and he underwent fusion with an allogenous bone graft. Satisfactory correction of the sagittal and coronal plane deformity was achieved, reducing the scoliosis to 14 degrees . At the 5-year follow-up, results of a clinical examination showed a marked improvement in truncal balance and walking ability. The patient had a rapidly progressive scoliosis and severe decompensation requiring surgical stabilization. The scoliosis behaved in a manner similar to that of neuromuscular scoliosis. Therefore, more aggressive treatment was warranted to prevent decompensation. For that reason, posterior long segment (T2-L4) pedicle screw instrumentation and fusion was performed for surgical treatment.