title: Unsuccessful intraventricular pentosan polysulphate treatment of variant Creutzfeldt-Jakob disease.
abstract: Pentosan polysulphate, delivered by chronic intraventricular infusion, has been proposed as a potential therapy for human prion disease. The first treated patient is still alive several years after treatment started. Here we describe in detail a case of variant Creutzfeldt-Jakob disease in which this treatment was started at a relatively early stage but had no definite clinical benefit. The patient died from disease progression 16 months after diagnosis and 5 months after pentosan polysulphate treatment was commenced.
authors.affiliation: Department of Clinical Neurosciences and National CJD Surveillance Unit, Western General Hospital, Edinburgh, UK. ian.whittle@ed.ac.uk
authors.forename: I R
authors.initials: IR
authors.lastname: Whittle
authors.forename: R S G
authors.initials: RS
authors.lastname: Knight
authors.forename: R G
authors.initials: RG
authors.lastname: Will
mesh_terms: D000328:Adult; D000405:Akinetic Mutism / Q000209:etiology / Q000503:physiopathology; D001706:Biopsy; D001921:Brain / Q000187:drug effects* / Q000473:pathology* / Q000503:physiopathology; D003072:Cognition Disorders / Q000209:etiology / Q000503:physiopathology; D007562:Creutzfeldt-Jakob Syndrome / Q000188:drug therapy* / Q000503:physiopathology / Q000523:psychology; D018450:Disease Progression; D004305:Dose-Response Relationship, Drug; D004791:Enzyme Inhibitors / Q000008:administration & dosage; D017809:Fatal Outcome; D005260:Female; D020234:Gait Ataxia / Q000209:etiology / Q000503:physiopathology; D006801:Humans; D015918:Infusion Pumps, Implantable; D007276:Injections, Intraventricular; D008279:Magnetic Resonance Imaging; D001523:Mental Disorders / Q000209:etiology / Q000503:physiopathology; D014066:Palatine Tonsil / Q000378:metabolism / Q000503:physiopathology; D010426:Pentosan Sulfuric Polyester / Q000008:administration & dosage*; D011328:Prions / Q000032:analysis / Q000378:metabolism; D012640:Seizures / Q000209:etiology / Q000503:physiopathology; D017211:Treatment Failure
chemical_list: D004791:Enzyme Inhibitors; D011328:Prions; D010426:Pentosan Sulfuric Polyester