title: Diagnosis of atlantoaxial subluxation in Morquio's syndrome and spondyloepiphyseal dysplasia congenita.
abstract: Compression of the spinal cord due to atlantoaxial subluxation was diagnosed in a patient with Morquio's syndrome and in another with spondyloepiphyseal dysplasia (SED) congenita by cervical radiography and magnetic resonance imaging (MRI). The patient with Morquio's syndrome, a 15 year old boy, had no neurologic symptoms and his somatosensory evoked potential (SSEP) was normal. However, MRI demonstrated spinal cord compression at C1-C2. In contrast, the patient with SED congenita, an 11 year old girl, had neck pain, hyperreflexia and loss of vibration sense in both legs. These findings were explained by the absence of P3 and later waves in SSEP and by compression of the spinal cord observed on MRI. Both SSEP and MRI should be used for evaluating disorders in which atlantoaxial subluxation might be present.
authors.affiliation: Department of Pediatrics, School of Medicine, University of Tokushima, Japan.
authors.forename: E
authors.initials: E
authors.lastname: Takeda
authors.forename: T
authors.initials: T
authors.lastname: Hashimoto
authors.forename: M
authors.initials: M
authors.lastname: Tayama
authors.forename: M
authors.initials: M
authors.lastname: Miyazaki
authors.forename: E
authors.initials: E
authors.lastname: Shirakawa
authors.forename: Y
authors.initials: Y
authors.lastname: Shiino
authors.forename: T
authors.initials: T
authors.lastname: Saijo
authors.forename: M
authors.initials: M
authors.lastname: Ito
authors.forename: E
authors.initials: E
authors.lastname: Naito
authors.forename: A H
authors.initials: AH
authors.lastname: Huq
mesh_terms: D000293:Adolescent; D001268:Atlanto-Axial Joint / Q000000981:diagnostic imaging* / Q000473:pathology; D002648:Child; D005073:Evoked Potentials, Somatosensory; D005260:Female; D006801:Humans; D004204:Joint Dislocations / Q000150:complications / Q000175:diagnosis* / Q000000981:diagnostic imaging; D008279:Magnetic Resonance Imaging; D008297:Male; D009085:Mucopolysaccharidosis IV / Q000150:complications*; D010009:Osteochondrodysplasias / Q000150:complications*; D011859:Radiography; D013117:Spinal Cord Compression / Q000175:diagnosis* / Q000209:etiology / Q000503:physiopathology