New method accurately detects prions in blood
A sensitive blood test accurately detected variant Creutzfeldt-Jakob disease, an incurable and fatal neurodegenerative disorder. The method could be used to diagnose prion diseases and prevent disease transmission.
CONCLUSIONS: The indistinguishable clinical features of P102L GSS patients with sCJD, especially in the early stage, support the importance of PRNP testing for diagnosing GSS. PMID: 30877692 [PubMed - as supplied by publisher]
enbaum Kuru, the first human prion disease was transmitted to chimpanzees by D. Carleton Gajdusek (1923–2008). In this review, we summarize the history of this seminal discovery, its anthropological background, epidemiology, clinical picture, neuropathology, and molecular genetics. We provide descriptions of electron microscopy and confocal microscopy of kuru amyloid plaques retrieved from a paraffin-embedded block of an old kuru case, named Kupenota. The discovery of kuru opened new vistas of human medicine and was pivotal in the subsequent transmission of Creutzfeldt–Jakob disease, as well as the ...
g J Abstract PMID: 30840102 [PubMed - as supplied by publisher]
CONCLUSIONS: The surveillance of CJD in ES (2004-2016) showed an increased incidence of CJD in ES, reaching the incidence rate of the whole country, with a permanent proportion of 70% gCJD cases based on the E200K mutation. Clinical, electrophysiological and MR features of sCJD and gCJD cases were in conformity with already published data. Epidemiological analysis of CJD in ES shows increasing detection of CJD but also suggests that current routine surveillance systems for CJD may underestimate the true burden of disease, especially sporadic cases in Slovakia. PMID: 30817871 [PubMed - in process]
Officials are worried that a prion disease spreading among deer in the U.S. may someday threaten the health of humans. Chronic wasting disease (CWD), which is nicknamed “zombie deer disease,” gets its name from a range of symptoms. For deer, elk and moose, these include stumbling, listlessness, drooling and rapid weight loss. The fatal disease degrades the brains, spines and bodies of the animals it infects, and there are no known treatments or vaccines. No cases of CWD have been recorded in humans. It has not even been definitely proven that humans can be infected by CWD, though some research has suggested it&...
A 71-year-old man was referred to Neuro-Ophthalmology for evaluation of reading problems associated to pituitary adenoma. Perimetry showed a right temporal crescent syndrome ( “half moon” syndrome) suggesting left occipital disease. Brain MRI, electroencephalogram and CSF examination (14-3-3 protein) were consistent with a diagnosis of Creutzfeldt-Jakob disease. His neurologic condition rapidly deteriorated with memory problems, myoclonus and apathy followed by death, four months later. This case shows how visual problems may be the first manifestation of this rare prion disease (Heidehain variant of Creutzfeldt-Jakob disease).
The disease course of dementia with Lewy bodies (DLB) can be rapidly progressive, clinically resembling Creutzfeldt-Jakob's disease (CJD). To better understand factors contributing to this rapidly progressive disease course, we describe load and distribution of neuropathology, and the presence of possible disease-associated genetic defects in a post-mortem series of DLB cases clinically suspected of CJD.
Tony Gibson, 33, from Lebanon, Tennessee, was diagnosed with Creutzfeldt-Jakob disease in May 2018. After being given one year to live, he passed away at a nursing home on January 30.
Poland has reported a case of atypical bovine spongiform encephalopathy (BSE) among cattle on a farm near the Czech border, the Paris-based World Organisation for Animal Health (OIE) said on Monday.