title: Cervical myelopathy in hereditary multiple exostoses.
abstract: Spinal cord compression due to cervical exostoses is a rare but recognized complication of hereditary multiple exostosis (HME), an autosomal dominant disorder. This disease, also called multiple osteochondromatosis, is characterised by osteocartilaginous exostoses, typically involving the juxtaepiphyseal regions of long bones. Complications such as transformation to sarcoma (1 to 5%) or neurological compression (of the spinal cord, 1 to 9%) can arise during the course of the disease. We report the case of a 64-year-old man with progressive difficulties in walking over many years, ascribed to congenital rachitism. A diagnosis of HME was not made until late in the disease course. Investigations revealed cervical myelopathy due to vertebral exostosis as well as multiple exostoses in other sites. His gait was not improved after surgical decompression. A better knowledge of this disease could have prevented this neurological complication.
authors.affiliation: Service de rhumatologie, département de l'appareil locomoteur, CHU Vaudois, Lausanne, Switzerland.
authors.forename: Vincent
authors.initials: V
authors.lastname: Burki
authors.forename: Alexander
authors.initials: A
authors.lastname: So
authors.forename: Bérengère
authors.initials: B
authors.lastname: Aubry-Rozier
mesh_terms: D002574:Cervical Vertebrae; D005097:Exostoses, Multiple Hereditary / Q000150:complications* / Q000473:pathology; D020233:Gait Disorders, Neurologic / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D013117:Spinal Cord Compression / Q000150:complications* / Q000473:pathology / Q000503:physiopathology; D013118:Spinal Cord Diseases / Q000150:complications* / Q000473:pathology; D014807:Vitamin D / Q000008:administration & dosage; D014808:Vitamin D Deficiency / Q000150:complications / Q000188:drug therapy
chemical_list: D014807:Vitamin D