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Intraventricular pentosan polysulphate in human prion diseases: an observational study in the UK
Author(s) -
Bone I.,
Belton L.,
Walker A. S.,
Darbyshire J.
Publication year - 2008
Publication title -
european journal of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.881
H-Index - 124
eISSN - 1468-1331
pISSN - 1351-5101
DOI - 10.1111/j.1468-1331.2008.02108.x
Subject(s) - medicine , observational study , intraventricular hemorrhage , natural history , anesthesia , pregnancy , biology , genetics , gestational age
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 μ g/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.