Intraventricular pentosan polysulphate in human prion diseases: an observational study in the UK

Eur J Neurol. 2008 May;15(5):458-64. doi: 10.1111/j.1468-1331.2008.02108.x. Epub 2008 Mar 18.


BACKGROUND, PURPOSE AND METHODS: This observational study assessed the effect of continuous intraventricular infusion of pentosan polysulphate (PPS) in seven patients at different clinical centres in the UK.

Results: Complications of intraventricular catheterization were frequent. PPS was well-tolerated over a wide dose range (11-110 microg/kg/day) during the 6-month study. Four patients were assessed for the entire study period: one remained stable, two showed minimal deterioration and one progressed significantly.

Conclusion: Mean survival of all patients was longer than reported values for natural history of specific prion disorders. Possible reasons for these findings are explored.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Anticoagulants / administration & dosage*
  • Female
  • Follow-Up Studies
  • Humans
  • Injections, Intraventricular / methods
  • Magnetic Resonance Imaging / methods
  • Male
  • Middle Aged
  • Neurologic Examination / methods
  • Observation
  • Pentosan Sulfuric Polyester / administration & dosage*
  • Prion Diseases / drug therapy*
  • Prion Diseases / mortality
  • Prion Diseases / pathology
  • Prion Diseases / surgery
  • Treatment Outcome
  • United Kingdom


  • Anticoagulants
  • Pentosan Sulfuric Polyester