Abstract
A woman, aged 59 years, underwent a cortical biopsy that led to the diagnosis of Creutzfeldt-Jakob disease (CJD). A man, aged 46 years, underwent cranial surgery in the same department 3 days later for brain contusion, with an uneventful recovery. Twenty six months later, he developed clinical signs of CJD with a typical EEG pattern. Both cases exhibited features of the 'ataxic' form of the disease with depletion of cerebellar granule cells, without kuru plaques or PrP deposits. PrP deposits were immuno-histochemically observed in the cerebrum, spinal cord and peripheral nerve. Molecular genetic analysis performed on brain tissue revealed the codon 129 polymorphism to be Met129Met in the donor and Met129Val in the recipient. The shared 'cerebellar' phenotype and the genotypic discrepancy between the two patients lead us to postulate that the 'cerebellar' agent strain plays a major role in CJD phenotype and transmission.
References
Dec 21, 1978·The New England Journal of Medicine·A G Dickinson, D M Taylor
Jun 15, 1991·Lancet·J CollingeA J Dryden
Jul 25, 1991·Nature·M S PalmerJ Collinge
Nov 1, 1988·Journal of Neurosurgery·V ThadaniJ W Prichard
Oct 5, 1988·Neuroscience Letters·D AllsopG G Glenner
Mar 21, 1974·The New England Journal of Medicine·D A Neumann
Jul 26, 1968·Science·C J GibbsW B Matthews
Apr 9, 1982·Science·S B Prusiner
Dec 1, 1994·Neurology·J L LaplancheJ M Launay
Apr 1, 1994·Neuropathology and Applied Neurobiology·T Billette de VillemeurO Robain
Feb 1, 1994·Molecular Neurobiology·D C Gajdusek
Jan 1, 1994·The Journal of General Virology·J P DeslysD Dormont
May 1, 1994·Annals of Neurology·P BrownD C Gajdusek
Feb 1, 1994·Neurology·P BrownC Masullo
Jan 23, 1996·Proceedings of the National Academy of Sciences of the United States of America·B GhettiF Tagliavini
Apr 6, 1996·Lancet·R G WillP G Smith
Oct 24, 1996·Nature·J CollingeA F Hill
Aug 1, 1965·Journal of Neurology, Neurosurgery, and Psychiatry·B BROWNELL, D R OPPENHEIMER
Citations
Dec 11, 2007·American Journal of Infection Control·Jane D SiegelUNKNOWN Health Care Infection Control Practices Advisory Committee
Mar 29, 2008·AORN Journal·Shauna Ely Tarrac
Apr 9, 2015·Infection Control and Hospital Epidemiology : the Official Journal of the Society of Hospital Epidemiologists of America·Paul Brown, Michael Farrell
Jun 24, 2014·Infection, Genetics and Evolution : Journal of Molecular Epidemiology and Evolutionary Genetics in Infectious Diseases·Stéphane Haïk, Jean-Philippe Brandel
Jun 3, 2016·Infectious Diseases of Poverty·Cao Chen, Xiao-Ping Dong
Jul 2, 2016·Neurosurgical Focus·David J BondaIgnazio Cali
Aug 17, 2016·Journal of Neurochemistry·Cathryn L UgaldeAndrew F Hill
Nov 2, 2017·Euro Surveillance : Bulletin Européen Sur Les Maladies Transmissibles = European Communicable Disease Bulletin·Fernando J García LópezJesús de Pedro-Cuesta
Jan 23, 2015·Journal of Virology·Atsushi KobayashiTetsuyuki Kitamoto
May 20, 1999·AORN Journal·V M Steelman
Oct 14, 2020·Biomedical Instrumentation & Technology·Camila Q M BrunaKazuko U Graziano
Nov 17, 2020·Frontiers in Bioengineering and Biotechnology·Hasier ErañaJoaquín Castilla
Dec 12, 2020·Viruses·Kristina JeonValerie L Sim
Apr 4, 2021·International Journal of Molecular Sciences·Hideyuki HaraSuehiro Sakaguchi