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Creutzfeldt–Jakob disease (CJD), also known as subacute spongiform encephalopathy or neurocognitive disorder due to prion disease, is a fatal neurodegenerative disease. 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 induces misfolding in normal variants of the same protein, leading to cellular death. Prions are responsible for prion diseases, known as transmissible spongiform encephalopathies (TSEs), which are fatal and transmissible neurodegenerative diseases affecting both humans and animals. These proteins can misfold sporadically, due to genetic mutations, or by exposure to an already misfolded protein, leading to an abnormal three-dimensional structure that can propagate misfolding in other proteins.
Fatal insomnia is an extremely rare neurodegenerative prion disease that results in trouble sleeping as its hallmark symptom. The majority of cases are familial, stemming from a mutation in the PRNP gene, with the remainder of cases occurring sporadically. The problems with sleeping typically start out gradually and worsen over time. Eventually, the patient will succumb to total insomnia, most often leading to other symptoms such as speech problems, coordination problems, and dementia. It results in death within a few months to a few years, and there is no known disease-modifying treatment.
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, it has been found to be experimentally transmissible to humanised transgenic mice and non-human primates.
Transmissible spongiform encephalopathies (TSEs), also known as prion diseases, are a group of progressive, incurable, 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 which may result in memory loss, personality changes, and abnormal or impaired movement which worsen over time.
Chronic wasting disease (CWD), sometimes called zombie deer disease, is a transmissible spongiform encephalopathy (TSE) affecting deer. TSEs are a family of diseases thought to be caused by misfolded proteins called prions and include similar diseases such as BSE in cattle, Creutzfeldt–Jakob disease (CJD) in humans and scrapie in sheep. Natural infection causing CWD affects members of the deer family. In the United States, CWD affects mule deer, white-tailed deer, red deer, sika deer, elk, bison, squirrels, antelope, caribou, and moose. The transmission of CWD to other species such as squirrel monkeys and humanized mice has been observed in experimental settings.
Encephalopathy means any disorder or disease of the brain, especially chronic degenerative conditions. In modern usage, encephalopathy does not refer to a single disease, but rather to a syndrome of overall brain dysfunction; this syndrome has many possible organic and inorganic causes.
Chorea is an abnormal involuntary movement disorder, characterized by quick movements of the hands or feet. It is one of a group of neurological disorders called dyskinesias. The term chorea is derived from the Ancient Greek: χορεία, as the movements of the body is comparable to dancing.
Gerstmann–Sträussler–Scheinker syndrome (GSS) is an extremely rare, always fatal neurodegenerative disease that affects patients from 20 to 60 years in age. It is exclusively heritable, and is found in only a few families all over the world. It is, however, classified with the transmissible spongiform encephalopathies (TSE) due to the causative role played by PRNP, the human prion protein. GSS was first reported by the Austrian physicians Josef Gerstmann, Ernst Sträussler and Ilya Scheinker in 1936.
Feline spongiform encephalopathy (FSE) is a neurodegenerative disease that affects the brains of felines. This disease is known to affect domestic, captive, and wild species of the family Felidae. Like BSE, this disease can take several years to develop. It is currently believed that this condition is a result of felines ingesting bovine meat contaminated with BSE.
Transmissible mink encephalopathy (TME) is a rare sporadic disease that affects the central nervous system of ranch-raised adult mink. It is a transmissible spongiform encephalopathy, caused by proteins called prions.
Variant Creutzfeldt–Jakob disease (vCJD), formerly known as New variant Creutzfeldt–Jakob disease (nvCJD) and referred to colloquially 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 prion disease which leads to tremors and loss of coordination from neurodegeneration.
Bovine spongiform encephalopathy (BSE), commonly known as mad cow disease, 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 World Health Organization 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.
The brain, like most other internal organs, or offal, can serve as nourishment. Brains used for nourishment include those of pigs, squirrels, rabbits, horses, cattle, monkeys, chickens, camels, fish, lamb, and goats. In many cultures, different types of brain are considered a delicacy.
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.
In biology, a pathogen, in the oldest and broadest sense, is any organism or agent that can produce disease. A pathogen may also be referred to as an infectious agent, or simply a germ.
Cerebellar degeneration is a condition in which cerebellar cells, otherwise known as neurons, become damaged and progressively weaken in the cerebellum. There are two types of cerebellar degeneration; paraneoplastic cerebellar degeneration, and alcoholic or nutritional cerebellar degeneration. As the cerebellum contributes to the coordination and regulation of motor activities, as well as controlling equilibrium of the human body, any degeneration to this part of the organ can be life-threatening. Cerebellar degeneration can result in disorders in fine movement, posture, and motor learning in humans, due to a disturbance of the vestibular system. This condition may not only cause cerebellar damage on a temporary or permanent basis, but can also affect other tissues of the central nervous system, those including the cerebral cortex, spinal cord and the brainstem.
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.
References
- ↑ "maghrebemergent.info Deux chercheurs algériens découvrent la maladie du " dromadaire fou " à Ouargla". Archived from the original on 2018-05-24. Retrieved 2018-05-27.
- ↑ "huffpostmaghreb.com Deux chercheurs algériens découvrent la maladie du "chameau fou" à Ouargla". Archived from the original on 2018-05-24. Retrieved 2018-05-27.
{{cite web}}: CS1 maint: unfit URL (link) - 1 2 Babelhadj, B; Di Bari, MA; Pirisinu, L; Chiappini, B; Gaouar, SBS; Riccardi, G; Marcon, S; Agrimi, U; Nonno, R; Vaccari, G (June 2018). "Prion Disease in Dromedary Camels, Algeria". Emerging Infectious Diseases. 24 (6): 1029–1036. doi:10.3201/eid2406.172007. PMC 6004840 . PMID 29652245.
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