Acquired human prion diseases - the past and the present issues

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  • HAGIWARA Ken'ichi
    Department of Biochemistry and Cell Biology,National Institute of Infectious Diseases
  • YAMAKAWA Yoshio
    Department of Biochemistry and Cell Biology,National Institute of Infectious Diseases
  • HANADA Kentaro
    Department of Biochemistry and Cell Biology,National Institute of Infectious Diseases

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Other Title
  • ヒト・プリオン病　‐　感染症としての変遷と新たな課題
  • ヒト・プリオン病--感染症としての変遷と新たな課題
  • ヒト プリオンビョウ カンセンショウ ト シテ ノ ヘンセン ト アラタ ナ カダイ

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Abstract

Transmissible spongiform encephalopathies, or prion diseases, are fatal neurodegenerative disorders. In aetiological viewpoint, human prion diseases are classified into 1) sporadic Creutzfeldt-Jakob disease (CJD) which comprises 80-90% of the total population of human prion disaeses, 2) inherited forms, and 3) acquired types by prion-contaminated surgical instruments, biopharmaceuticals or foodstuffs. The diseases cause an accumulation of the disease-associated form(s) of prion protein (PrPSc) in the central nervous system. PrPSc is regarded as the entity of prion agents and generally exerts infectivity, irrespective of its origin being from the sporadic cases or the inherited cases. Variant CJD (vCJD), first identified in the United Kingdom (UK) in 1996, is an acquired type of human CJD by oral intake of BSE prion. Cumulative numbers of 215 patients in the world have been reported for definite or probable vCJD cases according to the UK National Creutzfeldt-Jakob Disease Surveillance Unit by September, 2009. Different from sporadic CJD cases, vCJD patients show an accumulation of PrPSc in spleen and tonsils. Such distribution of PrPSc in lymphoid tissues raised clinical concern about the potential infectivity in the blood or blood components used for blood transfusion. To date, five instances of probable transfusion-mediated transmission of vCJD prion have been found in UK. Here we review the past and the present issues about the acquired human prion diseases.

Journal

  • Uirusu

    Uirusu 59 (2), 155-166, 2009

    The Japanese Society for Virology

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