Surgical management of neurological manifestations of mucopolysaccharidosis disorders

Tord D. Alden*, Hernán Amartino, Amauri Dalla Corte, Christina Lampe, Paul R. Harmatz, Leonardo Vedolin

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

24 Scopus citations


The mucopolysaccharidosis (MPS) disorders are ultra-rare lysosomal storage disorders associated with progressive accumulation of glycosaminoglycans (GAGs) in cells and tissues throughout the body. Clinical manifestations and progression rates vary widely across and within the different types of MPS. Neurological symptoms occur frequently, and may result directly from brain damage caused by infiltration of GAGs, or develop secondary to somatic manifestations such as spinal cord compression, hydrocephalus, and peripheral nerve entrapment. Management of secondary neurological manifestations often requires surgical correction of the underlying somatic cause. The present review discusses the surgical management of neurological disease in patients with MPS, including diagnostic imaging. Background information is derived from presentations and discussions during a meeting on the brain in MPS, attended by an international group of experts (April 28–30, 2016, Stockholm, Sweden), and additional literature searches.

Original languageEnglish (US)
Pages (from-to)41-48
Number of pages8
JournalMolecular Genetics and Metabolism
StatePublished - Dec 2017


  • Diagnostic imaging
  • Hydrocephalus
  • Mucopolysaccharidosis
  • Neurological disease
  • Spinal cord compression
  • Surgery

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism
  • Biochemistry
  • Molecular Biology
  • Genetics
  • Endocrinology


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