DiseasesChemicals and DrugsPsychiatry and PsychologyDisciplines and OccupationsAnthropology, Education, Sociology and Social PhenomenaGeographicals
KuruCannibalismPapua New GuineaFolkloreCreutzfeldt-Jakob SyndromeNew GuineaPrionsMortuary PracticeGerstmann-Straussler-Scheinker Disease
Kuru
A prion disease found exclusively among the Fore linguistic group natives of the highlands of NEW GUINEA. The illness is primarily restricted to adult females and children of both sexes. It is marked by the subacute onset of tremor and ataxia followed by motor weakness and incontinence. Death occurs within 3-6 months of disease onset. The condition is associated with ritual cannibalism, and has become rare since this practice has been discontinued. Pathologic features include a noninflammatory loss of neurons that is most prominent in the cerebellum, glial proliferation, and amyloid plaques. (From Adams et al., Principles of Neurology, 6th ed, p773)
Cannibalism
Eating other individuals of one's own species.
Papua New Guinea
A country consisting of the eastern half of the island of New Guinea and adjacent islands, including New Britain, New Ireland, the Admiralty Islands, and New Hanover in the Bismarck Archipelago; Bougainville and Buka in the northern Solomon Islands; the D'Entrecasteaux and Trobriand Islands; Woodlark (Murua) Island; and the Louisiade Archipelago. It became independent on September 16, 1975. Formerly, the southern part was the Australian Territory of Papua, and the northern part was the UN Trust Territory of New Guinea, administered by Australia. They were administratively merged in 1949 and named Papua and New Guinea, and renamed Papua New Guinea in 1971.
Folklore
The common orally transmitted traditions, myths, festivals, songs, superstitions, and stories of all peoples.
Creutzfeldt-Jakob Syndrome
A rare transmissible encephalopathy most prevalent between the ages of 50 and 70 years. Affected individuals may present with sleep disturbances, personality changes, ATAXIA; APHASIA, visual loss, weakness, muscle atrophy, MYOCLONUS, progressive dementia, and death within one year of disease onset. A familial form exhibiting autosomal dominant inheritance and a new variant CJD (potentially associated with ENCEPHALOPATHY, BOVINE SPONGIFORM) have been described. Pathological features include prominent cerebellar and cerebral cortical spongiform degeneration and the presence of PRIONS. (From N Engl J Med, 1998 Dec 31;339(27))
New Guinea
Originally an island of the Malay Archipelago, the second largest island in the world. It divided, West New Guinea becoming part of Indonesia and East New Guinea becoming Papua New Guinea.
Prions
Small proteinaceous infectious particles which resist inactivation by procedures that modify NUCLEIC ACIDS and contain an abnormal isoform of a cellular protein which is a major and necessary component. The abnormal (scrapie) isoform is PrPSc (PRPSC PROTEINS) and the cellular isoform PrPC (PRPC PROTEINS). The primary amino acid sequence of the two isoforms is identical. Human diseases caused by prions include CREUTZFELDT-JAKOB SYNDROME; GERSTMANN-STRAUSSLER SYNDROME; and INSOMNIA, FATAL FAMILIAL.
Mortuary Practice
Activities associated with the disposition of the dead. It excludes cultural practices such as funeral rites.
Gerstmann-Straussler-Scheinker Disease
An autosomal dominant familial prion disease with a wide spectrum of clinical presentations including ATAXIA, spastic paraparesis, extrapyramidal signs, and DEMENTIA. Clinical onset is in the third to sixth decade of life and the mean duration of illness prior to death is five years. Several kindreds with variable clinical and pathologic features have been described. Pathologic features include cerebral prion protein amyloidosis, and spongiform or neurofibrillary degeneration. (From Brain Pathol 1998 Jul;8(3):499-513; Brain Pathol 1995 Jan;5(1):61-75)

Unusual resistance to ionizing radiation of the viruses of kuru, Creutzfeldt-Jakob disease, and scrapie. (1/40)

The titers of several preparations of kuru. Creutzfeldt-Jacob disease, and scrapie viruses were reduced by only 1/10th or less by high doses of gamma radiation of 50 kGy and by only 1/10th-1/1000th or less for 200 kGy. This unusual radiation resistance of the two human viruses further links them with the scrapie virus and suggests that the genetic information of all three viruses is considerably smaller than that of any other known viruses of mammals.  (+info)

Genetic influence on the structural variations of the abnormal prion protein. (2/40)

Prion diseases are characterized by the presence of the abnormal prion protein PrP(Sc), which is believed to be generated by the conversion of the alpha-helical structure that predominates in the normal PrP isoform into a beta-sheet structure resistant to proteinase K (PK). In human prion diseases, two major types of PrP(Sc), type 1 and 2, can be distinguished based on the difference in electrophoretic migration of the PK-resistant core fragment. In this study, protein sequencing was used to identify the PK cleavage sites of PrP(Sc) in 36 cases of prion diseases. We demonstrated two primary cleavage sites at residue 82 and residue 97 for type 1 and type 2 PrP(Sc), respectively, and numerous secondary cleavages distributed along the region spanning residues 74-102. Accordingly, we identify three regions in PrP(Sc): one N-terminal (residues 23-73) that is invariably PK-sensitive, one C-terminal (residues 103-231) that is invariably PK-resistant, and a third variable region (residues 74-102) where the site of the PK cleavage, likely reflecting the extent of the beta-sheet structure, varies mostly as a function of the PrP genotype at codon 129.  (+info)

Increased susceptibility to Kuru of carriers of the PRNP 129 methionine/methionine genotype. (3/40)

Kuru reached epidemic proportions by the mid-twentieth century among the Fore people of New Guinea and disappeared after the abolition of cannibalistic rituals. To determine susceptibility to kuru and its role in the spread and elimination of the epidemic, we analyzed the PRNP gene coding sequences in 5 kuru patients; no germline mutations were found. Analysis of the PRNP 129 methionine (M)/valine (V) polymorphism in 80 patients and 95 unaffected controls demonstrated that the kuru epidemic preferentially affected individuals with the M/M genotype. A higher representation of M/M carriers was observed among the affected young Fore males entering the age of risk, whereas a lower frequency of M/M homozygotes was found among the survivors. M/V and V/V genotypes predisposed to a lower risk of disease development and longer incubation times. These findings are relevant to the current outbreak of variant Creutzfeldt-Jakob disease (vCJD) in the United Kingdom, because all vCJD patients tested thus far have been M/M carriers.  (+info)

Genetic studies in relation to Kuru. VI. Evaluation of increased liability to Kuru in Gc Ab-Ab individuals. (4/40)

The validity of the reported association between GcAb and kuru is analyzed. Phenotypes with one or more GcAb genes have an increased incidence of the disease at the expense of Gc 1-1 and Gc 2-2. Incidence ratios of kuru associated with various phenotypes examined over the linguistic groups studied indicate that only Gc Ab-Ab persons have a significantly greater chance of dying of kuru. The association X2 for the incidence ratio for those phenotypes possessing only one GcAb gene is significant, but there is significant heterogeneity between groups studied. Those of the Gc Ab-Ab phenotype are six times as likely to contract kuru as the baseline group. Criticisms of this analysis include difficulties defining an adequate control group in such heterogeneous populations, errors in determination of Gc phenotypes, inclusion of persons incubating kuru in the control groups, and questions of validity of statistical tests in isolated inbred populations.  (+info)

Prion diseases: a dual view of the prion hypothesis as seen from a distance. (5/40)

We review the historical background and principles of the prion theory in its current shape. We showed that most of data may be still interpreted dually according to the protein only hypothesis and according to the theory in which additional component is necessary to comprise the infectivity. The enormous impact of structural biological studies is also stressed.  (+info)

Balancing selection at the prion protein gene consistent with prehistoric kurulike epidemics. (6/40)

Kuru is an acquired prion disease largely restricted to the Fore linguistic group of the Papua New Guinea Highlands, which was transmitted during endocannibalistic feasts. Heterozygosity for a common polymorphism in the human prion protein gene (PRNP) confers relative resistance to prion diseases. Elderly survivors of the kuru epidemic, who had multiple exposures at mortuary feasts, are, in marked contrast to younger unexposed Fore, predominantly PRNP 129 heterozygotes. Kuru imposed strong balancing selection on the Fore, essentially eliminating PRNP 129 homozygotes. Worldwide PRNP haplotype diversity and coding allele frequencies suggest that strong balancing selection at this locus occurred during the evolution of modern humans.  (+info)

Human evolution: a legacy of cannibalism in our genes? (7/40)

A new study of genetic variation in the human prion protein gene suggests that balancing selection has operated on an amino acid sequence polymorphism in the gene during the last five hundred thousand years. Is this a legacy of widespread cannibalism by our ancestors?  (+info)

Creutzfeldt-Jakob disease with congophilic kuru plaques: CT and pathological findings of the cerebral white matter. (8/40)

In a patient whose Creutzfeldt-Jakob disease with congophilic kuru plaques that was proved at necropsy, the early brain CT showed low-density areas in the cerebral white matter before cortical atrophy and ventricular enlargement became apparent. Subsequently, there occurred diffuse white matter lucency and severe brain atrophy. At necropsy, there was severe white matter destruction which was more prominent than cortical neuronal loss. Serial CT scans were of great value for demonstrating the early and predominant changes in the cerebral white matter.  (+info)

Kuru is a degenerative, fatal neurological disorder once prevalent among the Fore tribe in Papua New Guinea, characterized by tremors, muscle jerks, and progressive loss of motor control, caused by prions (infectious proteins) found in human brain tissue.

Kuru is a rare, fatal neurological disorder that was identified in the Fore people of Papua New Guinea. It is primarily caused by an abnormal form of protein called prion and is transmitted through cannibalistic practices where infected human tissues are consumed. The disease is characterized by progressive deterioration of the brain, leading to symptoms such as tremors, difficulty coordinating movements, slurred speech, and uncontrollable laughter. There is currently no known cure for kuru, and it has become extremely rare due to the cessation of cannibalistic rituals in the affected population.

Cannibalism is the act or practice of consuming all or part of another individual of the same species as food, which in humans would be considered a rare psychological disorder and illegal in most societies.

Cannibalism is defined in medical terms as the act or practice of consuming flesh or organs of one's own species as food. It is a term that is often used to describe situations where humans consume the flesh or organs of other humans. Cannibalism can occur in various contexts, including survival situations, cultural practices, and criminal activities.

It is important to note that cannibalism is generally considered taboo in most societies and cultures today. In medical and psychological terms, cannibalism can be associated with a range of negative consequences, such as the transmission of infectious diseases, ethical concerns, and psychological distress. However, it is essential to approach this topic with sensitivity and cultural relativism, recognizing that cultural practices and beliefs may vary widely across different societies and historical periods.

I'm sorry for any confusion, but "Papua New Guinea" is not a medical term and does not have a medical definition; it is the name of a country located in the southwest Pacific Ocean, near Australia and Indonesia, which consists of the eastern half of the island of New Guinea and numerous offshore islands.

I'm sorry for any confusion, but "Papua New Guinea" is not a medical term or concept. It is the name of a country located in the southwest Pacific Ocean, made up of the eastern half of the island of New Guinea and numerous offshore islands. If you have any questions about medical topics or definitions, I would be happy to help with those!

Folklore, in a medical context, refers to the use of traditional beliefs, stories, and practices related to health and healing within a cultural group, which may or may not have scientific validation.

I'm afraid there seems to be a misunderstanding. Folklore is not a medical term and does not have a medical definition. It refers to the traditional customs, tales, sayings, dances, or art forms that are passed down from generation to generation within a culture or community. If you have any questions related to medical terminology or health-related topics, I'd be happy to help!

Creutzfeldt-Jakob Syndrome (CJD) is a rapidly progressive, incurable neurodegenerative disorder characterized by rapid cognitive decline, motor dysfunction, and distinctive neuropathological findings including widespread vacuolation and deposition of prion protein scrapie isoform (PrPSc) in the brain, which is typically transmitted sporadically, familially, or iatrogenically.

Creutzfeldt-Jakob syndrome (CJD) is a rare, degenerative, and fatal brain disorder. It is caused by an abnormal form of protein called prion that can cause normal proteins in the brain to fold into abnormal shapes and accumulate, leading to damage and death of brain cells.

The symptoms of CJD usually develop over a period of several months and include rapidly progressing dementia, memory loss, confusion, coordination problems, muscle stiffness, twitching, and shaking. Some people may also experience visual hallucinations, changes in personality, or depression.

There are three main types of CJD: sporadic, inherited, and acquired. Sporadic CJD is the most common form and accounts for about 85% of all cases. It occurs spontaneously with no known cause. Inherited CJD is caused by a genetic mutation that is passed down from parents to their children. Acquired CJD is caused by exposure to contaminated tissue or bodily fluids, such as through a medical procedure or eating contaminated beef (variant CJD).

There is no cure for Creutzfeldt-Jakob syndrome and it is fatal, usually within a year of onset of symptoms. Treatment focuses on managing the symptoms and making the patient as comfortable as possible.

New Guinea is the world's second largest island, located in the southwest Pacific, shared by Papua New Guinea (the eastern half) and Indonesia (the western half, known as Western New Guinea or Papua).

I'm sorry for any confusion, but "New Guinea" is not a medical term. It is the second largest island in the world, located in the southwest Pacific Ocean, northeast of Australia. The island is divided politically between the independent nation of Papua New Guinea to the east and the Indonesian provinces of Papua and West Papua to the west.

If you have any medical questions or terms that you would like defined, I'd be happy to help!

Prions are abnormally folded proteinaceous particles that lack nucleic acid, which can induce other normal proteins to adopt their pathological conformation, leading to neurodegenerative diseases.

Prions are misfolded proteins that can induce other normal proteins to also adopt the misfolded shape, leading to the formation of aggregates. These abnormal prion protein aggregates are associated with a group of progressive neurodegenerative diseases known as transmissible spongiform encephalopathies (TSEs). Examples of TSEs include bovine spongiform encephalopathy (BSE or "mad cow disease") in cattle, variant Creutzfeldt-Jakob disease (vCJD) in humans, and scrapie in sheep. The misfolded prion proteins are resistant to degradation by proteases, which contributes to their accumulation and subsequent neuronal damage, ultimately resulting in spongiform degeneration of the brain and other neurological symptoms associated with TSEs.

'Mortuary practice' is a term used to describe the professional procedures and techniques involved in the handling, preparation, and preservation of deceased human bodies for purposes such as medical examination, identification, or funeral rites.

Mortuary practice, also known as mortuary science or funeral service, is a field that deals with the handling, preparation, and disposal of dead human bodies. This can include tasks such as:

1. The removal and transportation of the body from the place of death to the mortuary.
2. The cleaning and sanitization of the body.
3. The reconstruction of the body, if necessary, to make it presentable for viewing.
4. The application of cosmetics to restore a natural appearance to the deceased.
5. The dressing and casketing of the body.
6. The coordination of funeral services, such as memorial services or viewings.
7. The completion of necessary paperwork, such as death certificates and burial permits.

Mortuary practitioners may work in a variety of settings, including hospitals, funeral homes, and coroner's offices. They must have a strong understanding of anatomy, physiology, and infection control, as well as excellent communication and interpersonal skills to provide support and guidance to grieving families.

It is important to note that mortuary practices can vary depending on cultural, religious, and personal beliefs, so practitioners must be respectful and sensitive to the needs and wishes of each family they serve.

Gerstmann-Straussler-Scheinker disease is a rare, rapidly progressive, and universally fatal autosomal dominant neurodegenerative disorder characterized by cerebellar ataxia, pyramidal signs, and distinctive spongiform changes in the brain resulting from prion protein accumulation.

Gerstmann-Straussler-Scheinker disease (GSS) is a rare, inherited, progressive neurodegenerative disorder characterized by cerebellar ataxia, pyramidal signs, and distinctive histopathological features in the brain. It is caused by mutations in the PRNP gene, which encodes the prion protein. The disease is transmitted in an autosomal dominant pattern, meaning that a single copy of the mutated gene from either parent is sufficient to cause the disorder.

GSS typically begins in mid-adulthood and progresses over several years to a decade, leading to severe disability and death. The symptoms of GSS include cerebellar ataxia (difficulty with coordination and balance), pyramidal signs (stiffness, spasticity, and hyperreflexia in the limbs), and various other neurological features such as dementia, visual disturbances, and speech difficulties.

Histopathologically, GSS is characterized by the accumulation of abnormal prion protein aggregates in the brain, which can be detected using special staining techniques. These aggregates are thought to be responsible for the neurodegeneration and clinical symptoms of the disease. Currently, there is no cure for GSS and treatment is focused on managing the symptoms of the disorder.

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