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


Prof. Ian Bone, Department of Medical and Cardiovascular Studies, Western Infirmary, Glasgow G11 6NT, UK (tel.: 44 1436 671472 e-mail:


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 μg/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.