A moderately severe spasticity affecting all four limbs, but especially the legs and the right side, developed in a 36-year-old man with mucopolysaccharidosis II (Hunter syndrome) who for 6 months had suffered from pain in the neck radiating into the shoulder. Myelography, computed tomography and magnetic resonance imaging revealed thickening of the cervical meninges as the cause of the symptoms. A laminectomy was performed and the foramen magnum enlarged by an occipital craniotomy. At operation dorsal thickening of the dura was evident; thickened tissue was also palpable in front of the dural sac. Histological examination of a tissue sample revealed deposition of acid mucopolysaccharides. The gait of the patient was improved after the operation and the neck pain disappeared. Similar findings were present in other family members.
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