Progressive spastic paraplegia: the combination of Scheuermann's disease, a short-segmented kyphosis and dysplastic thoracic spinous processes.

Abstract

Case report. To describe a rare case of paraplegia in a patient with Scheuermann's disease and dysplastic thoracic spinous processes. Spinal Cord Injury Center, Orthopaedic University Hospital Heidelberg, Heidelberg, Germany. The authors report on a 15-year-old boy with progressive incomplete spastic paraplegia presenting segmental dysplastic thoracic spinous processes and Scheuermann's disease. The magnetic resonance imaging showed a kyphotic angulation at T 5/6 and signs of myelopathy. Hypoplastic thoracic processes and hypoplastic paraspinal muscles in the upper thoracic spine were observed intraoperatively. In this case, dorsoventral stabilization from T 4-7 was performed and the neurological outcome improved at follow-up (6 months). Paraplegia can be accelerated in patients with Scheuermann's disease, severe kyphotic angulation and dysplastic posterior elements. After operative treatment, neurological recovery and a normal walking pattern were shown.