[Cervical myelopathy in mucopolysaccharidosis type II (Hunter's syndrome). Neuroradiologic, clinical and histopathologic findings]. / Zervikale Myelopathie bei Mucopolysaccharidose Type II (Hunter-Syndrom). Neuroradiologische, klinische und histopathologische Befunde.

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.

Polyclonal antibodies against iduronate 2-sulphate sulphatase from human urine.

Human iduronate 2-sulphate sulphatase (EC 3.1.6.-) from urine has been purified by affinity chromatography on concanavalin A-Sepharose, ammonium sulphate fractionation and DEAE-cellulose chromatography. With ion-exchange chromatography, the enzyme was resolved in two activity peaks. The less anionic of these forms was further purified by polyacrylamide gel electrophoresis under non-denaturing conditions. Anti-iduronate 2-sulphate sulphatase antibodies were obtained from mice immunized with polyacrylamide eluted enzyme. The specificity of the antibodies towards iduronate 2-sulphate sulphatase was demonstrated by immunoprecipitation of the enzyme from partially purified urine protein. The procedure described in this work opens the way to the application of hybridoma technology to iduronate 2-sulphate sulphatase.