SYMPOSIUM: KURU: 50 YEARS. Transmissible spongiform encephalopathies or prion diseases – update 2007
Paweł P. Liberski, Jolanta Bratosiewicz-Wąsik, Herbert Budka, James W. Ironside, Beata Sikorska
Affiliation and address for correspondenceAktualn Neurol 2007, 7 (3), p. 158-187
Abstract
Prion diseases or transmissible spongiform encephalopathies are a group of neurodegenerative disorders characterized by a widespread deposition in the central nervous system, and some other tissues, of the pathological isoform of the prion protein (PrPd; “d” from disease). TSEs include:
1) kuru;
2) Creutzfeldt-Jakob disease (CJD), the disease that occurs in four etiologically forms:
• sporadic CJD (sCJD),
• familial CJD (fCJD),
• iatrogenic CJD (iCJD),
• variant CJD (vCJD);
3) Gerstmann-Sträussler-Scheinker disease (GSS);
4) fatal familial insomnia (FFI).
There are also several TSEs in animals:
– scrapie – in sheep, goats and moufflons;
– bovine spongiform encephalopathy (BSE) and bovine atypical spongiform encephalopathy (BASE) or mad cow disease;
– BSE passaged to:
• domestic cats (feline spongiform encephalopathy, FSE) and large cats (lion, tiger, cheetah, ocelot and puma)
• antelopes (kudu, nyala, oryx), bison;
– chronic wasting disease in mule deer and elk;
– transmissible mink encephalopathy in ranch-reared mink.
Historically, the eponimic names are also included: atactic type of CJD or Betty Brownell syndrome, CJD with a cortical blindness (Heidenhain type) and the panencephalopathic type (Tateishi). The amyotrophic type is not TSE but motor neuron disease with dementi. In 1996, a new variant CJD (now only vCJD) was discovered as a result of a passage from BSE to humans.
Keywords
transmissible spongiform encephalopathies, prions, kuru, Creutzfeldt-Jakob disease, review
