Cerebroventricular infusion of pentosan polysulphate in human variant Creutzfeldt–Jakob disease

Variant CJD

“…Studies in animal models raised the possibility that pentosan polysulphate (PPS) might be a candidate treatment for vCJD 101 and extended survival has been reported in a small number of treated cases. 102,103 However, this medication has to be given by intraventricular infusion, requiring a neurosurgical procedure, and treated patients continued to decline with no reversal of severe neurological deficits. 104 Postmortem examination of one case of vCJD treated with PPS showed extensive and severe pathology.…”

Potent Antiscrapie Activities of Degenerate Phosphorothioate Oligonucleotides

“…However, because PPS does not effectively cross the blood-brain barrier, it must be injected into the brain to be beneficial once the infection has reached the central nervous system. Orally dosed PPS (Elmiron) is a Food and Drug Administrationapproved treatment for interstitial cystitis, and PPS is now being evaluated as a treatment for CJD in humans by the use of direct dosing into the brain (42).…”

Inhibition of Protease-Resistant Prion Protein Formation in a Transformed Deer Cell Line Infected with Chronic Wasting Disease

“…Using this cell line, we have identified the first two inhibitors of PrP CWD formation, pentosan polysulfate (PPS) and indium (III) meso-tetra (4-sulfonatophenyl)porphine chloride (In-TSP). PPS is a well known anti-TSE compound in other experimental models and is currently being tested to treat human CJD patients (39). In-TSP is a newly identified inhibitor and a member of the well-established cyclic tetrapyrrole class of anti-TSE compounds (11,30,31).…”