Neurosurgical interventions in children with Maroteaux-Lamy syndrome. Case report and review of the literature

V I Vougioukas; A Berlis; M V Kopp; R Korinthenberg; J Spreer; V van Velthoven

doi:10.1159/000050383

Neurosurgical interventions in children with Maroteaux-Lamy syndrome. Case report and review of the literature

Pediatr Neurosurg. 2001 Jul;35(1):35-8. doi: 10.1159/000050383.

Authors

V I Vougioukas¹, A Berlis, M V Kopp, R Korinthenberg, J Spreer, V van Velthoven

Affiliation

¹ Department of Neurosurgery, University of Freiburg, Germany. vvougio@nz.ukl.uni-freiburg.de

PMID: 11490189
DOI: 10.1159/000050383

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.

Publication types

Case Reports
Review

MeSH terms

Adolescent
Cervical Vertebrae / diagnostic imaging
Cervical Vertebrae / pathology
Cervical Vertebrae / surgery
Decompression, Surgical
Female
Humans
Hydrocephalus / diagnosis
Hydrocephalus / etiology
Hydrocephalus / surgery
Laminectomy
Magnetic Resonance Imaging
Mucopolysaccharidosis VI / complications
Mucopolysaccharidosis VI / diagnosis
Mucopolysaccharidosis VI / surgery*
Optic Atrophy / etiology
Optic Atrophy / pathology
Optic Atrophy / surgery
Paraparesis, Spastic / diagnosis
Paraparesis, Spastic / etiology
Paraparesis, Spastic / surgery
Spinal Cord Compression / etiology
Spinal Cord Compression / surgery
Syndrome
Tomography, X-Ray Computed