title: [Cervical myelopathy in mucopolysaccharidosis type II (Hunter's syndrome). Neuroradiologic, clinical and histopathologic findings].
abstract: 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.
authors.affiliation: Abteilung für Neuroradiologie, Krankenhaus Nordwest, Klinikum der Universität Frankfurt.
authors.forename: S
authors.initials: S
authors.lastname: Kaendler
authors.forename: S
authors.initials: S
authors.lastname: Bockenheimer
authors.forename: H
authors.initials: H
authors.lastname: Gräfin Vitzthum
authors.forename: W
authors.initials: W
authors.lastname: Galow
mesh_terms: D000328:Adult; D004388:Dura Mater / Q000473:pathology; D006801:Humans; D007066:Iduronate Sulfatase / Q000652:urine; D007796:Laminectomy; D008279:Magnetic Resonance Imaging; D008297:Male; D016532:Mucopolysaccharidosis II / Q000150:complications* / Q000000981:diagnostic imaging / Q000235:genetics / Q000473:pathology / Q000601:surgery; D009192:Myelography; D009333:Neck; D013116:Spinal Cord / Q000000981:diagnostic imaging; D013118:Spinal Cord Diseases / Q000000981:diagnostic imaging / Q000209:etiology* / Q000235:genetics / Q000473:pathology / Q000601:surgery; D014057:Tomography, X-Ray Computed
chemical_list: D007066:Iduronate Sulfatase
vernacular_title: Zervikale Myelopathie bei Mucopolysaccharidose Type II (Hunter-Syndrom). Neuroradiologische, klinische und histopathologische Befunde.