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 microg/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.
journal_name
Acta Neurol Scandjournal_title
Acta neurologica Scandinavicaauthors
Terada T,Tsuboi Y,Obi T,Doh-ura K,Murayama S,Kitamoto T,Yamada T,Mizoguchi Kdoi
10.1111/j.1600-0404.2009.01272.xsubject
Has Abstractpub_date
2010-02-01 00:00:00pages
127-30issue
2eissn
0001-6314issn
1600-0404pii
ANE1272journal_volume
121pub_type
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