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Less protease‐resistant PrP in a patient with sporadic CJD treated with intraventricular pentosan polysulphate
Author(s) -
Terada T.,
Tsuboi Y.,
Obi T.,
Dohura K.,
Murayama S.,
Kitamoto T.,
Yamada T.,
Mizoguchi K.
Publication year - 2010
Publication title -
acta neurologica scandinavica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.967
H-Index - 95
eISSN - 1600-0404
pISSN - 0001-6314
DOI - 10.1111/j.1600-0404.2009.01272.x
Subject(s) - intraventricular hemorrhage , medicine , protease , prion protein , disease , anesthesia , gastroenterology , surgery , pathology , biology , enzyme , pregnancy , biochemistry , genetics , gestational age
Terada T, Tsuboi Y, Obi T, Doh‐ura K, Murayama S, Kitamoto T, Yamada T, Mizoguchi K. Less protease‐resistant PrP in a patient with sporadic CJD treated with intraventricular pentosan polysulphate.
Acta Neurol Scand: 2010: 121: 127–130.
© 2009 The Authors Journal compilation © 2009 Blackwell Munksgaard. 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 μg/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.