NR AVEY
AU Whittle,I.R.; Knight,R.S.G.; Will,R.G.
TI Unsuccessful intraventricular pentosan polysulphate treatment of variant Creutzfeldt-Jakob disease
QU Acta Neurochirurgica 2006 Jun; 148(6): 677-9; discussion 679
PT case reports; journal article
AB 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.
MH Adult; Akinetic Mutism/etiology/physiopathology; Biopsy; Brain/*drug effects/*pathology/physiopathology; Cognition Disorders/etiology/physiopathology; Creutzfeldt-Jakob Syndrome/*drug therapy/physiopathology/psychology; Disease Progression; Dose-Response Relationship, Drug; Enzyme Inhibitors/administration & dosage; Fatal Outcome; Female; Gait Ataxia/etiology/physiopathology; Humans; Infusion Pumps, Implantable; Injections, Intraventricular; Magnetic Resonance Imaging; Mental Disorders/etiology/physiopathology; Pentosan Sulfuric Polyester/*administration & dosage; Prions/analysis/metabolism; Seizures/etiology/physiopathology; Tonsil/metabolism/physiopathology; Treatment Failure
AD Department of Clinical Neurosciences and National CJD Surveillance Unit, Western General Hospital, Edinburgh, UK. ian.whittle@ed.ac.uk
SP englisch
PO Österreich