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

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

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1
Department of Medical and Cardiovascular Studies, Western Infirmary, Glasgow, UK. gcl136@clinmed.gla.ac.uk

Abstract

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.

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