Less protease-resistant PrP in a patient with sporadic CJD treated with intraventricular pentosan polysulphate

T. Terada, Y. Tsuboi, T. Obi, K. Doh-Ura, S. Murayama, T. Kitamoto, T. Yamada, K. Mizoguchi

Research output: Contribution to journalComment/debatepeer-review

21 Citations (Scopus)

Abstract

Treatment with intraventricular pentosan polysulphate (PPS) might be beneficial in patients with Creutzfeldt-Jakob disease. We report a 68-year-old woman with sporadic Creutzfeldt-Jakob disease who received continuous intraventricular PPS infusion (1-120 μg/kg/day) for 17 months starting 10 months after the onset of clinical symptoms. Treatment with PPS was well tolerated but was associated with a minor, transient intraventricular hemorrhage and a non-progressive collection of subdural fluid. The patient's overall survival time was well above the mean time expected for the illness but still within the normal range. Post-mortem examination revealed that the level of abnormal protease-resistant prion protein in the brain was markedly decreased compared with levels in brains without PPS treatment. These findings suggest that intraventricular PPS infusion might modify the accumulation of abnormal prion proteins in the brains of patients with sporadic Creutzfeldt-Jakob disease.

Original languageEnglish
Pages (from-to)127-130
Number of pages4
JournalActa Neurologica Scandinavica
Volume121
Issue number2
DOIs
Publication statusPublished - 2010 Feb

Keywords

  • Creutzfeldt-Jakob disease
  • Intraventricular infusion
  • Pentosan polysulphate
  • Prion protein

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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