Creutzfeldt–Jakob disease (CJD), also known as subacute spongiform encephalopathy or neurocognitive disorder due to prion disease, is an invariably fatal degenerative brain disorder. Early symptoms include memory problems, behavioral changes, poor coordination, and visual disturbances. Later symptoms include dementia, involuntary movements, blindness, weakness, and coma. About 70% of people die within a year of diagnosis. The name Creutzfeldt–Jakob disease was introduced by Walther Spielmeyer in 1922, after the German neurologists Hans Gerhard Creutzfeldt and Alfons Maria Jakob.
A prion is a misfolded protein that can transmit its misfolded shape onto normal variants of the same protein. Prions are the causative agent of prion diseases known as transmissible spongiform encephalopathies (TSEs) that are transmissible, fatal neurodegenerative diseases in humans and other animals. The proteins may misfold sporadically, due to genetic mutations, or by exposure to an already misfolded protein. The consequent abnormal three-dimensional structure confers them the ability to cause misfolding of other proteins.
Scrapie is a fatal, degenerative disease affecting the nervous systems of sheep and goats. It is one of several transmissible spongiform encephalopathies (TSEs), and as such it is thought to be caused by a prion. Scrapie has been known since at least 1732 and does not appear to be transmissible to humans. However, new studies suggest a link between scrapie and sporadic CJD.
Transmissible spongiform encephalopathies (TSEs) also known as prion diseases, are a group of progressive and fatal conditions that are associated with prions and affect the brain and nervous system of many animals, including humans, cattle, and sheep. According to the most widespread hypothesis, they are transmitted by prions, though some other data suggest an involvement of a Spiroplasma infection. Mental and physical abilities deteriorate and many tiny holes appear in the cortex causing it to appear like a sponge when brain tissue obtained at autopsy is examined under a microscope. The disorders cause impairment of brain function, including memory changes, personality changes and problems with movement that worsen chronically.
Feline spongiform encephalopathy (FSE) is a disease that affects the brains of felines. It is caused by proteins called prions. FSE is thought to be related or identical to bovine spongiform encephalopathy (BSE). This disease is known to affect domestic and captive feline species. This infectious agent might be spread by both haematogenous and nervous pathways. Like BSE, this disease can take several years to develop. It is probable, but not proven, that the affected animals contract the disease by eating contaminated bovine meat.
Major prion protein(PrP), is encoded in the human by the PRNP gene also known as CD230 (cluster of differentiation 230). Expression of the protein is most predominant in the nervous system but occurs in many other tissues throughout the body.
John Mark Purdey was an English organic farmer who came to public attention in the 1980s, when he began to circulate his own theories regarding the causes of bovine spongiform encephalopathy.
Variant Creutzfeldt–Jakob disease (vCJD), commonly referred to as "mad cow disease" or "human mad cow disease" to distinguish it from its BSE counterpart, is a fatal type of brain disease within the transmissible spongiform encephalopathy family. Initial symptoms include psychiatric problems, behavioral changes, and painful sensations. In the later stages of the illness, patients may exhibit poor coordination, dementia and involuntary movements. The length of time between exposure and the development of symptoms is unclear, but is believed to be years to decades. Average life expectancy following the onset of symptoms is 13 months.
Kuru is a rare, incurable, and fatal neurodegenerative disorder that was formerly common among the Fore people of Papua New Guinea. Kuru is a form of transmissible spongiform encephalopathy (TSE) caused by the transmission of abnormally folded proteins (prions), which leads to symptoms such as tremors and loss of coordination from neurodegeneration.
Bovine spongiform encephalopathy (BSE), is an incurable and invariably fatal neurodegenerative disease of cattle. Symptoms include abnormal behavior, trouble walking, and weight loss. Later in the course of the disease the cow becomes unable to function normally. There is conflicting information about the time between infection and onset of symptoms. In 2002, the WHO suggested it to be approximately four to five years. Time from onset of symptoms to death is generally weeks to months. Spread to humans is believed to result in variant Creutzfeldt–Jakob disease (vCJD). As of 2018, a total of 231 cases of vCJD had been reported globally.
Meat and bone meal (MBM) is a product of the rendering industry. It is typically about 48–52% protein, 33–35% ash, 8–12% fat, and 4–7% water. It is primarily used in the formulation of animal feed to improve the amino acid profile of the feed. Feeding of MBM to cattle is thought to have been responsible for the spread of BSE ; therefore, in most parts of the world, MBM is no longer allowed in feed for ruminant animals. However, it is still used to feed monogastric animals.
The National Prion Clinic (UK) is part of the University College London Hospitals NHS Foundation Trust. Its aim is to diagnose and treat patients with any form of human prion disease (Creutzfeldt-Jakob disease, CJD). In addition, the clinic facilitates research in diagnostics and therapeutics, organises clinical trials, and counsels those with an increased genetic risk of the disease.
Dundee Cell Products (DCP) is a biotechnology company headquartered in Dundee, Scotland, United Kingdom.
Surround optical-fiber immunoassay (SOFIA) is an ultrasensitive, in vitro diagnostic platform incorporating a surround optical-fiber assembly that captures fluorescence emissions from an entire sample. The technology's defining characteristics are its extremely high limit of detection, sensitivity, and dynamic range. SOFIA's sensitivity is measured at the attogram level (10−18 g), making it about one billion times more sensitive than conventional diagnostic techniques. Based on its enhanced dynamic range, SOFIA is able to discriminate levels of analyte in a sample over 10 orders of magnitude, facilitating accurate titering.
Rosalind Ridley is a British psychologist and researcher who was head of the Medical Research Council Comparative Cognition Research Team in the Department of Psychology, Cambridge, UK, until 2005. She was a fellow of Newnham College, Cambridge from 1995–2010 and Vice-Principal from 2000–2005. She holds the privileges of a Fellow Emerita at Newnham College.
Frank O. Bastian is an American medical doctor and neuropathologist, who previously worked at Louisiana State University, moved to a university in New Orleans in 2019. He specializes in the transmissible spongiform encephalopathies (TSEs), which include, but are not limited to, Bovine spongiform encephalopathy (BSE) "Mad cow disease" in cattle, scrapie in sheep and goats, and Creutzfeldt–Jakob disease (CJD) in humans.
Diseases of abnormal polymerization, or simply DAPs, are a class of disorders characterized by a novel alteration in base unit proteins that results in a structure with pathogenic potential. This functional alteration in a protein in relation to its thermodynamic and kinetic properties enacts an extended chain response among neighboring proteins until an extensive and potentially harmful polymerized structure is formed. Due to this endogenous foreign formation, these diseases are often untreatable and very severe in clinical manifestation. Although DAPs are rare infections, the poor outcome in patients and the need for further understanding makes this class of diseases a pillar for future research.
The United Kingdom was afflicted with an outbreak of Bovine spongiform encephalopathy, and its human equivalent variant Creutzfeldt–Jakob disease (vCJD), in the 1980s and 1990s. Over four million head of cattle were slaughtered in an effort to contain the outbreak, and 178 people died after contracting vCJD through eating infected beef. A political and public health crisis resulted, and British beef was banned from export to numerous countries around the world, with some bans remaining in place until as late as 2019.
Real-time quaking-induced conversion (RT-QuIC) is a highly sensitive assay for prion detection.
Michael Coulthart is a Canadian microbiologist and sometime pathologist who is employed as the head of the Canadian Creutzfeldt–Jakob Disease Surveillance System (CJDSS) within the Public Health Agency of Canada (PHAC), which terms CJD a zoonotic and infectious disease. In 2006, a working group named "classic CJD" as well as Variant Creutzfeldt–Jakob disease as two notifiable diseases. It is unknown whether PHAC tracks in an official capacity other transmissible spongiform encephalopathies (TSE), but Coulthart is on the Advisory Committee of the Center for Infectious Disease Research and Policy for Chronic Wasting Disease of cervidae.
