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-...

BACKGROUND Creutzfeldt-Jakob disease (CJD) in humans and chronic wasting disease (CWD) in deer and elk occur in the United States. Recent reports of 3 unusually young patients with CJD who regularly consumed deer or elk meat created concern about the possible zoonotic transmission of CWD. OBJECTIVE To examine the possible transmission of CWD to humans. PATIENTS Three unusually young patient...

Isolates from six patients with Creutzfeldt-Jakob disease (CJD) were injected into various strains of hamsters and mice, and the infective agent was propagated. Serially passaged cultures were established from these CJD agent-infected brains and from uninfected control brains. All healthy cultures (21 out of 21) from CJD agent-infected brains became immortal and/or transformed. In contrast only...

Creutzfeldt-Jakob disease (CJD) in humans and bovine spongiform encephalopathy (BSE) in cattle are subacute degenerative diseases of the brain classified as transmissible spongiform encephalopathies. BSE was first identified in 1986 in the United Kingdom (UK), where an epizootic involving > 155,000 cattle appeared to have been greatly amplified by exposure of calves to contaminated rendered cat...

Creutzfeldt-Jakob disease (CJD) is a rare and fatal neurodegenerative disorder with a worldwide incidence of 1-1.5 per million. As in other countries, a CJD surveillance unit with a clinical and neuropathological approach was established in Goettingen (Germany) in 1993. Here we report the epidemiological data from a prospective 12-year surveillance. Since 1993, there has been an increasing inci...

Although neuropathological examination is still required for the definite diagnosis of Creutzfeldt-Jakob disease (CJD), specialised clinical assessment predicts probable CJD. Here we present a 73-year-old female patient presenting with rapid cognitive decline, visual, acoustic and cerebellar disturbances, ataxia and EEG changes compatible with early CJD stages. MRI revealed hyperintensities wit...