Acquired prion disease: iatrogenic CJD, variant CJD, kuru

Variant CJD

Abbreviations: BSE, bovine spongiform encephalopathy; CWD, chronic wasting disease; GSS, Gerstman–Str€aussler–Scheinker disease; M, methionine; PPS, pentosan polysulphate; PrP, protease-resistant prion protein; PrP, abnormal prion protein; QuIC, quaking-induced conversion; TSE, transmissible spongiform encephalopathy; V, valine; vCJD, variant Creutzfeldt–Jakob disease; VPSPr, variably protease-...

CJD and prion disease.indd

Human Prion Protein V129 Prevents Expression of Variant CJD Phenotype

Materials and Methods Transgenic mice. Transgenic mice homozygous for a human PrP 129V transgene array and murine PrP null alleles (Prnp) designated Tg(HuPrP129V Prnp)-152 mice (129VV Tg152 mice) and transgenic mice homozygous for a human PrP 129M transgene array and murine PrP null alleles (Prnp) designated Tg(HuPrP129M Prnp)-35 mice (129MM Tg35 mice) have been described previously (S1-S4). In...

The origin of the prion agent of kuru: molecular and biological strain typing

Kuru is an acquired human prion disease that primarily affected the Fore linguistic group of the Eastern Highlands of Papua New Guinea. The central clinical feature of kuru is progressive cerebellar ataxia and, in sharp contrast to most cases of sporadic Creutzfeldt-Jakob disease (CJD), dementia is a less prominent and usually late clinical feature. In this regard, kuru is more similar to varia...

The neuropsychology of variant CJD: a comparative study with inherited and sporadic forms of prion disease.

OBJECTIVE To assess cognitive function in variant Creutzfeldt-Jakob disease (vCJD). We describe the neuropsychological profiles of 10 cases and compare these data with cross sectional data obtained from patients with histologically confirmed sporadic CJD and cases with inherited prion disease with confirmed mutations in the prion protein gene. METHODS Patients referred to the Specialist Cogni...