MRI of the brain and craniocervical junction in Morquio's disease

• Published in: Neuroradiology Vol. 39; no. 5; pp. 381 - 385
• Main Authors: HUGHES, D. G, CHADDERTON, R. D, COWIE, R. A, WRAITH, J. E, JENKINS, J. P. R
• Format: Journal Article
• Language: English
• Published: Berlin Springer 01.05.1997
• Subjects: Adolescent; Biological and medical sciences; Brain - pathology; Cervical Vertebrae - pathology; Child; Child, Preschool; Female; Humans; Infant; Investigative techniques, diagnostic techniques (general aspects); Magnetic Resonance Imaging; Male; Medical sciences; Mucopolysaccharidosis IV - complications; Mucopolysaccharidosis IV - diagnosis; Osteoarticular system. Muscles; Radiodiagnosis. Nmr imagery. Nmr spectrometry; Spinal Cord - pathology; Spinal Cord Compression - diagnosis; Spinal Cord Compression - etiology; Human; Radiodiagnosis; Diseases of the osteoarticular system; Metabolic diseases; Spine disease; Enzymopathy; Nuclear magnetic resonance imaging; Genetic disease; Morquio disease; Young adult; Medical imagery; Skull; Carbohydrate; Diagnosis; Preoperative; Child; Cervical spine
• ISSN: 0028-3940; 1432-1920
• DOI: 10.1007/s002340050429

We reviewed MRI of the brain and cervical spine in 11 patients with Morquio's disease. No abnormality was seen in the brain. The odontoid peg was abnormal in all patients, with varying degrees of cord compression due to an anterior soft tissue mass and indentation by the posterior arch of the atlas. The degree of cord compression was more marked than suggested by the symptoms and signs. We recommend MRI of the cervical spine in children with Morquio's disease before the development of neurological symptoms, to optimise the timing and type of surgical intervention.