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 Maroteux-Lamy syndrome are discussed and reviewed.

Keywords:
Maroteaux-Lamy syndrome, Mucopolysaccharidosis, Optic degeneration, Cervical myelopathy, Hydrocephalus
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© 2001 S. Karger AG, Basel
2001
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