Less protease-resistant PrP in a patient with sporadic CJD treated with intraventricular pentosan polysulphate.
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.
Department of Neurology, Shizuoka Institute of Epilepsy and Neurological Disorders, Shizuoka, Japan.
T
T
Terada
Y
Y
Tsuboi
T
T
Obi
K
K
Doh-ura
S
S
Murayama
T
T
Kitamoto
T
T
Yamada
K
K
Mizoguchi
D000368:Aged; D001921:Brain / Q000000981:diagnostic imaging / Q000187:drug effects / Q000378:metabolism*; D007562:Creutzfeldt-Jakob Syndrome / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000378:metabolism*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D018696:Neuroprotective Agents / Q000008:administration & dosage / Q000627:therapeutic use*; D010426:Pentosan Sulfuric Polyester / Q000008:administration & dosage / Q000627:therapeutic use*; D011328:Prions / Q000378:metabolism*; D013997:Time Factors; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
D018696:Neuroprotective Agents; D011328:Prions; D010426:Pentosan Sulfuric Polyester