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UNLABELLED: 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.

Original publication

DOI

10.1111/j.1468-1331.2008.02108.x

Type

Journal article

Journal

Eur J Neurol

Publication Date

05/2008

Volume

15

Pages

458 - 464

Keywords

Adult, Anticoagulants, Female, Follow-Up Studies, Humans, Injections, Intraventricular, Magnetic Resonance Imaging, Male, Middle Aged, Neurologic Examination, Observation, Pentosan Sulfuric Polyester, Prion Diseases, Treatment Outcome, United Kingdom