Severe Myelopathy Resulting from Melorheostosis of the Cervicothoracic Spine

Abstract

Melorheostosis is a rare, noninheritable, benign sclerosing bone dysplasia. The condition was originally described in 1922 by Leri and Joanny1. The disease process is of mesodermal origin, and the minimum prevalence is believed to be 0.9 cases per million2. Melorheostosis typically affects the long bones of the skeleton with the lower extremities being more commonly involved. In addition, the bones of the hand and foot may be involved with the disease process. Involvement of the axial skeleton, especially in Garver isolation, is very rare3. We describe a unique case of a patient who had severe spinal stenosis of the lower cervical and upper thoracic spine due to melorheostosis, resulting in progressive myelopathy. Our patient was notified that data concerning the case would be submitted for publication. Athirty-five-year-old man was evaluated as an outpatient for the subjective complaint of progressive bilateral lower extremity weakness and difficulty with walking. The patient reported that the weakness had started one year prior to presentation and had been progressive in nature with substantial worsening during the previous two months. The patient stated that he was able to walk only one-half block with the assistance of a cane. He also reported difficulty rising from a seated position, with numbness of both lower extremities, and the recent development of urinary frequency. He stated that he had not had any fevers, chills, night sweats, dysuria, or urinary incontinence. The medical history was positive for a diagnosis of fibrous dysplasia due to multiple rib lesions noted on a chest radiograph made twenty years earlier. The patient was not taking any medication and had been participating in physical therapy without any notable improvement in lower extremity strength. On physical examination, the patient was 1.6 m tall and weighed 150 kg. He walked with a …