title: Hereditary multiple exostoses causing cord compression.
abstract: Hereditary multiple exostoses (HME) is an autosomal dominant skeletal disorder characterized by the presence of multiple osseous prominences. It can occur sporadically or within families (22 - 56%). Two genes, EXT1 and EXT2 located respectively at 8q24 and 11p11-p12, have been isolated to cause HME. It can cause gross deformity of limbs and growth disturbance which is quite a common complication. Malignant transformation to chondrosarcoma can also occur. Neurological presentations are rare and usually happened due to direct compression of a peripheral nerve or nerve root or less often the spinal cord. This case is possibly the first case of HME described from Bangladesh, presented with dorsal cord compression. Decompression was done and the complaints of myelopathy were improved.
authors.affiliation: Department of Medicine, Sylhet M.A.G. Osmani Medical College, Sylhet, Bangladesh.
authors.forename: Md Shafiqul
authors.initials: MS
authors.lastname: Bari
authors.forename: M M
authors.initials: MM
authors.lastname: Jahangir Alam
authors.forename: Fazle Rabbi
authors.initials: FR
authors.lastname: Chowdhury
authors.forename: Pulin Bihari
authors.initials: PB
authors.lastname: Dhar
authors.forename: Afsana
authors.initials: A
authors.lastname: Begum
mesh_terms: D000293:Adolescent; D019299:Decompression, Surgical; D005097:Exostoses, Multiple Hereditary / Q000150:complications* / Q000235:genetics / Q000473:pathology; D020233:Gait Disorders, Neurologic / Q000209:etiology / Q000473:pathology; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D013117:Spinal Cord Compression / Q000209:etiology* / Q000473:pathology / Q000601:surgery; D013904:Thoracic Vertebrae; D016896:Treatment Outcome