Thoracic myelopathy with alkaptonuria.

Abstract

A case of thoracic myelopathy with alkaptonuria (ochronotic spondyloarthropathy) is presented. To present and review the first reported case of an alkaptonuric patient with concomitant thoracic myelopathy. Alkaptonuria, a rare hereditary metabolic disease, is characterized by accumulation of homogentistic acid, ochronosis, and destruction of connective tissue resulting in degenerative spondylosis and arthritis. Despite the high incidence of intervertebral disc diseases among patients with alkaptonuria, neurologic symptoms caused by spinal disease are rare. Thoracic myelopathy in a patient with alkaptonuria has not been previously reported. The clinical course, radiologic features, pathology, and treatment outcome of an alkaptonuria patient with thoracic myelopathy was documented. Myelopathy of the patient was caused by rupture of a thoracic intervertebral disc. The neurologic symptoms of the patient were markedly improved after surgery. We have reported for the first time, that an alkaptonuria patient showed thoracic myelopathy caused by rupture of a thoracic intervertebral disc. Decompression followed by the instrumented fusion of the thoracic spine was effective for improving the neurologic symptoms.