MUCOPOLYSACCHARIDOSIS - THICKENING OF DURA-MATER AT THE CRANIOCERVICAL JUNCTION AND OTHER CT MRI FINDINGS/

Cranial CT and/or MRI imaging of 8 patients with mucopolysaccharidosis (MPS) was retrospectively evaluated. Two patients had MPS IH, 1 had M PS IS, 1 had MPS IVA and 4 had MPS IV CT and MRI showed thickening of dura mater at the cranio-cervical junction, causing narrowing of the s ubarachnoid space, in all the patients examined. Spinal cord compressi on was detected in 4 patients. Other findings were: white matter alter ations, mild to severe hydrocephalus, skull dysplasia and odontoid dys plasia. White matter alterations were evident as large areas and as mu ltiple dispersed spots of prolonged TI and T2 value. Reduced gray/whit e matter contrast was demonstrated on T2-weighted MRI images. It is im portant to examine the cranio-cervical junction carefully for thickeni ng of dura mater in all patients with mucopolysaccharidosis examined b y CT or MRI, because of the generally progressive clinical course of M PS. In patients with symptomatic cord compression, surgical interventi on should be considered.