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Long term survival in a patient with variant Creutzfeldt–Jakob disease treated with intraventricular pentosan polysulphate
  1. A Parry1,
  2. I Baker1,
  3. R Stacey1,
  4. S Wimalaratna2
  1. 1Department of Neurology, John Radcliffe Hospital, Headington, Oxford, UK
  2. 2Great Western Hospital, Swindon, UK
  1. Correspondence to:
 Dr A Parry
 Department of Neurology, John Radcliffe Hospital, Headley Way, Headington, Oxford OX3 9DU, UK; allysonparry{at}hotmail.com

Abstract

Variant Creutzfeldt-Jakob disease (vCJD) is a neurodegenerative disease that principally affects young people and has a median duration of illness of 13 (range 6–39) months. vCJD is incurable and there are currently no treatments that conclusively slow the rate of disease progression. However, recent animal studies and isolated case reports have suggested that treatment with intraventricular pentosan polysulphate (PPS) may be beneficial in the treatment of patients with vCJD.

We report a case of a 22-year-old male with vCJD treated 19 months after the onset of clinical symptoms with continuous intraventricular PPS (32 μg/kg/day) over a period of 31 months. Treatment with PPS appeared to be safe and well tolerated and was associated with prolonged survival (51 months) when compared to natural history studies. However, PPS treatment did not appear to arrest the progression of the disease.

  • PPS, pentosan polysulphate
  • vCJD, variant Creutzfeldt–Jakob disease

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Footnotes

  • Published Online First 21 February 2007

  • Competing interests: None.

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