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Cranial computed tomography (CT) was performed on 11 cases of mucopolysaccharidosis (MPS) IVA (Morquio syndrome). Our results suggest that although the patients may have normal intelligence CT changes may be seen with increasing age. In two cases white matter low density was found and in a third there was gross dilatation of the ventricles, basal cisterns and subarachnoid space. Nine other patients with various types of mucopolysaccharidosis also had cranial CT performed and in general those types associated with mental retardation showed changes although there was an interesting exception involving a case of MPS IIIA who had a normal CT scan.

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Lumbar myelography using water-soluble contrast media provides a simple means of assessing the patency of theco-peritoneal shunts. Our experience with two consecutive patients is described.