title: Neurosurgical interventions in children with Maroteaux-Lamy syndrome. Case report and review of the literature.
abstract: This paper reports the case of a 14-year-old child with Maroteaux-Lamy syndrome (mucopolysaccharidosis type 6) who was treated consecutively for compressive damage of the optic nerves, hydrocephalus communicans and progressive spastic tetraparesis within 2 years. The clinical course of the patient is presented and the pathophysiologic mechanisms of disease progression in patients with Maroteaux-Lamy syndrome are discussed and reviewed.
authors.affiliation: Department of Neurosurgery, University of Freiburg, Germany. vvougio@nz.ukl.uni-freiburg.de
authors.forename: V I
authors.initials: VI
authors.lastname: Vougioukas
authors.forename: A
authors.initials: A
authors.lastname: Berlis
authors.forename: M V
authors.initials: MV
authors.lastname: Kopp
authors.forename: R
authors.initials: R
authors.lastname: Korinthenberg
authors.forename: J
authors.initials: J
authors.lastname: Spreer
authors.forename: V
authors.initials: V
authors.lastname: van Velthoven
mesh_terms: D000293:Adolescent; D002574:Cervical Vertebrae / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery; D019299:Decompression, Surgical; D005260:Female; D006801:Humans; D006849:Hydrocephalus / Q000175:diagnosis / Q000209:etiology / Q000601:surgery; D007796:Laminectomy; D008279:Magnetic Resonance Imaging; D009087:Mucopolysaccharidosis VI / Q000150:complications / Q000175:diagnosis / Q000601:surgery*; D009896:Optic Atrophy / Q000209:etiology / Q000473:pathology / Q000601:surgery; D020336:Paraparesis, Spastic / Q000175:diagnosis / Q000209:etiology / Q000601:surgery; D013117:Spinal Cord Compression / Q000209:etiology / Q000601:surgery; D013577:Syndrome; D014057:Tomography, X-Ray Computed