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Record 1,  Textual support,  English

Record number: 1, Textual support number: 1 CONT

Chronic wasting disease(CWD) is a progressive, fatal nervous system disease that affects animals which are all part of the deer family[, and] known as cervids. It is a transmissible spongiform encephalopathy, or prion disease. It is contagious amongst cervids, like scrapie in sheep. There has been no known transmission of CWD to humans, however, bovine spongiform encephalopathy(also known as mad cow disease) in cattle has been known to cause Creutzfeldt-Jakob disease in humans.  6, record 1, English, - chronic%20wasting%20disease%20of%20cervids

Record number: 1, Textual support number: 1 OBS

... in deer, moose, elk, and caribou or reindeer.  7, record 1, English, - chronic%20wasting%20disease%20of%20cervids

Record 1,  Textual support,  French

Record number: 1, Textual support number: 1 CONT

La maladie débilitante chronique (MDC) est une maladie progressive et mortelle du système nerveux qui touche [les] animaux qui font [...] partie de la famille des cervidés. C'est une encéphalopathie spongiforme transmissible, aussi appelée maladie à prion. Elle est contagieuse chez les cervidés, comme la tremblante du mouton. Il n'y a pas eu de transmission connue de l'encéphalopathie des cervidés aux humains, mais on sait que l'encéphalopathie spongiforme bovine (aussi appelée maladie de la vache folle) chez les bovins cause la maladie de Creutzfeldt-Jakob chez les humains.  2, record 1, French, - maladie%20d%C3%A9bilitante%20chronique%20des%20cervid%C3%A9s

Record number: 1, Textual support number: 1 OBS

[...] chez les cerfs, les orignaux, les wapitis et les caribous.  1, record 1, French, - maladie%20d%C3%A9bilitante%20chronique%20des%20cervid%C3%A9s

Record 1,  Textual support,  Spanish

Record number: 1, Textual support number: 1 OBS

En ciervos y wapitíes.  2, record 1, Spanish, - caquexia%20cr%C3%B3nica

Record 2,  Textual support,  English

Record number: 2, Textual support number: 1 CONT

The diagnosis of a primary degenerative dementia can only be entertained after secondary dementias have been ruled out. Primary degenerative dementias include Alzheimer's disease, Lewy body dementia, frontotemporal dementia/Pick's disease, Parkinson's disease, Huntington's disease, and the prion diseases Creutzfeldt-Jakob disease and bovine spongiform encephalopathy.  2, record 2, English, - primary%20degenerative%20dementia

Record number: 2, Textual support number: 1 OBS

primary degenerative dementia: The word "dementia" is sometimes considered pejorative. However, there is no generally accepted synonym to replace the designation "primary degenerative dementia."  3, record 2, English, - primary%20degenerative%20dementia

Record 2,  Textual support,  French

Record number: 2, Textual support number: 1 OBS

démence dégénérative primaire : Le mot «démence» est parfois considéré comme péjoratif. Toutefois, il n'existe pas de synonyme généralement accepté pour remplacer la désignation «démence dégénérative primaire».  2, record 2, French, - d%C3%A9mence%20d%C3%A9g%C3%A9n%C3%A9rative%20primaire

Record 2,  Textual support,  Spanish

Record 3,  Textual support,  English

Record number: 3, Textual support number: 1 CONT

Prion diseases are rare, fatal, degenerative brain disorders that are thought to occur worldwide in both humans and animals.... Although there are several forms of human prion disease, the most common is Creutzfeldt-Jakob disease(CJD).... Prion diseases that affect animals include bovine spongiform encephalopathy(BSE) in cattle, scrapie in sheep and goats, and chronic wasting disease(CWD) in deer, moose, elk, and caribou or reindeer. Although they are rare, these diseases have caused great public health concern because of their many difficult and unusual features.  7, record 3, English, - transmissible%20subacute%20spongiform%20encephalopathy

Record 3,  Textual support,  French

Record number: 3, Textual support number: 1 CONT

Les maladies à prion sont des troubles dégénératifs rares et fatals du cerveau qui se produisent dans le monde entier chez les humains et les animaux. [...] Bien qu'il y ait plusieurs formes de maladies à prion [humaines], la plus courante est la maladie de Creutzfeldt-Jakob (MCJ). [...] Quant aux maladies à prion qui touchent les animaux, on compte l'encéphalopathie spongiforme bovine (ESB) chez les bovins, la tremblante du mouton chez les moutons et les chèvres, ainsi que la maladie débilitante chronique des cervidés (MDC) chez les cerfs, les orignaux, les wapitis et les caribous. Bien que ces maladies soient rares, elles ont causé de graves préoccupations en matière de santé publique en raison de leurs nombreuses caractéristiques inhabituelles et difficiles.  3, record 3, French, - enc%C3%A9phalopathie%20subaigu%C3%AB%20spongiforme%20transmissible

Record 3,  Textual support,  Spanish

Record 4,  Textual support,  English

Record number: 4, Textual support number: 1 DEF

A human prion disease clinically, biochemically, and pathologically linked to bovine spongiform encephalopathy, manifesting the features of CJD and due to infection with the same strain of prion.  8, record 4, English, - variant%20Creutzfeldt%2DJakob%20disease

Record 4,  Textual support,  French

Record number: 4, Textual support number: 1 CONT

On admet généralement que la nouvelle variante de la maladie de Creutzfeldt-Jakob est liée à la consommation d'aliments contenant des tissus de système nerveux central provenant de bovins atteints d'encéphalopathie spongiforme bovine.  2, record 4, French, - variante%20de%20la%20maladie%20de%20Creutzfeldt%2DJakob

Record 4,  Textual support,  Spanish

Record 5,  Textual support,  English

Record number: 5, Textual support number: 1 DEF

A human prion disease caused by a normal prion protein that becomes misshapen into an infectious prion, builds up in the brain and disrupts normal brain function.  2, record 5, English, - Creutzfeldt%2DJakob%20disease

Record number: 5, Textual support number: 1 OBS

It is a rare disorder that is fatal, usually within 6 months of diagnosis and a year of the first symptom.  2, record 5, English, - Creutzfeldt%2DJakob%20disease

Record number: 5, Textual support number: 2 OBS

A81.0: code used in the International Statistical Classification of Diseases and Related Health Problems.  3, record 5, English, - Creutzfeldt%2DJakob%20disease

Record 5,  Textual support,  French

Record number: 5, Textual support number: 1 CONT

Causée par l'accumulation de prions dans les tissus cérébraux, la MCJ est une maladie neurodégénérative dont on connaît aujourd'hui trois formes : familiale (génétique), sporadique (origine inconnue) et variante.  2, record 5, French, - maladie%20de%20Creutzfeldt%2DJakob

Record number: 5, Textual support number: 1 OBS

A81.0 : code de la Classification statistique internationale des maladies et des problèmes de santé connexes.  3, record 5, French, - maladie%20de%20Creutzfeldt%2DJakob

Record 5,  Textual support,  Spanish

Record 6,  Textual support,  English

Record 6,  Textual support,  French

Record number: 6, Textual support number: 1 OBS

Thème d'une rencontre internationale organisée par le ministère de la Santé les 5 et 6 juin 1996.  1, record 6, French, - La%20maladie%20de%20Creutzfeldt%2DJakob%20%3A%20la%20prise%20de%20d%C3%A9cisions%20en%20p%C3%A9riode%20d%27incertitude

Record 6,  Textual support,  Spanish

Record 7,  Textual support,  English

Record number: 7, Textual support number: 1 DEF

Inherited form of Creutzfeldt-Jakob disease which shows an autosomal dominant pattern of disease segregation associated with a gene mutation.  1, record 7, English, - familial%20Creutzfeldt%2DJakob%20disease

Record number: 7, Textual support number: 1 OBS

If onset is usually at a slightly younger age (± 50), it generally shows clinical and pathological features analogous to the sporadic form.  1, record 7, English, - familial%20Creutzfeldt%2DJakob%20disease

Record 7,  Textual support,  French

Record number: 7, Textual support number: 1 DEF

Forme héréditaire à transmission autosomique dominante de la maladie de Creutzfeldt-Jakob associée à une mutation du gène de la prion-protéine.  1, record 7, French, - maladie%20de%20Creutzfeldt%2DJakob%20familiale

Record number: 7, Textual support number: 1 OBS

Elle survient plus précocement que la forme sporadique (âge moyen : 50 ans). D'évolution plus longue, elle présente des caractéristiques cliniques et neuropathologiques analogues à la forme sporadique.  1, record 7, French, - maladie%20de%20Creutzfeldt%2DJakob%20familiale

Record 7,  Textual support,  Spanish

Record 8,  Textual support,  English

Record number: 8, Textual support number: 1 DEF

Subtype of Creutzfeldt-Jakob disease which results from the transmission by direct inoculation in the central nervous system of the causative agent via medical or surgical treatment using accidentally contaminated materials(surgical instruments, grafts, transplants).  1, record 8, English, - directly%20inoculated%20iatrogenic%20Creutzfeldt%2DJakob%20disease

Record 8,  Textual support,  French

Record number: 8, Textual support number: 1 DEF

Forme de la maladie de Creutzfeldt-Jakob, due à la transmission par inoculation directe dans le système nerveux central de l'agent infectieux lors d'un geste chirurgical ou neurochirurgical, par l'intermédiaire de transplants, d'homogreffes et d'instruments insuffisamment décontaminés.  1, record 8, French, - maladie%20de%20Creutzfeldt%2DJakob%20iatrog%C3%A8ne%20de%20proximit%C3%A9

Record 8,  Textual support,  Spanish

Record 9,  Textual support,  English

Record number: 9, Textual support number: 1 DEF

Subtype of Creutzfeldt-Jakob disease which results from the introduction by intravenous or intramuscular injection of contaminated human pituitary-derived preparations(growth hormone, gonadotrophin).  1, record 9, English, - peripherally%20acquired%20iatrogenic%20Creutzfeldt%2DJakob%20disease

Record 9,  Textual support,  French

Record number: 9, Textual support number: 1 DEF

Forme de la maladie de Creutzfeldt-Jakob due à la transmission par injection intraveineuse ou intramusculaire de l'agent infectieux lors de l'utilisation thérapeutique de dérivés hypophysaires d'origine humaine (hormone de croissance, gonadotrophine).  1, record 9, French, - maladie%20de%20Creutzfeldt%2DJakob%20iatrog%C3%A8ne%20p%C3%A9riph%C3%A9rique

Record 9,  Textual support,  Spanish

Record 10,  Textual support,  English

Record number: 10, Textual support number: 1 DEF

A subtype of Creutzfeldt-Jakob clinically characterized by a rapidly progressive dementia, behavioural changes with progression to neurological abnormalities and onset of a progressive cerebellar syndrome, and anatomically by microvacuolar spongiform changes in the cerebellar molecular layer accompanied by reactive gliosis and neuronal loss.  1, record 10, English, - sporadic%20Creutzfeldt%2DJakob%20disease

Record number: 10, Textual support number: 1 OBS

It arises spontaneously in individuals aged between 50 and 75 without any apparent cause.  1, record 10, English, - sporadic%20Creutzfeldt%2DJakob%20disease

Record 10,  Textual support,  French

Record number: 10, Textual support number: 1 DEF

Forme de la maladie de Creutzfeldt-Jakob caractérisée cliniquement par une détérioration mentale progressive, des troubles comportementaux neurologiques avec une nette prédominance cérébelleuse et, anatomiquement, par une microspongiose au niveau de la couche cellulaire, une réaction gliale avec astrocytose ainsi qu'une déperdition neuronale.  1, record 10, French, - maladie%20de%20Creutzfeldt%2DJakob%20sporadique

Record number: 10, Textual support number: 1 OBS

La cause de cette maladie, qui se déclare en moyenne vers l'âge de 60 ans et atteint les individus isolément, n'a pas encore été trouvée.  1, record 10, French, - maladie%20de%20Creutzfeldt%2DJakob%20sporadique

Record 10,  Textual support,  Spanish

Record 11,  Textual support,  English

Record number: 11, Textual support number: 1 CONT

Prions are the cause of certain diseases, including bovine spongiform encephalopathy, otherwise known as the ’mad cow disease’, and the Creutzfeldt-Jakob disease afflicting humans. They are still, however, a major biological puzzle. These "unconventional transmissible agents" afflicting humans as well as animals cause lethal degenerative diseases of the central nervous system in the bodies where they multiply. They do not respond to any known category of human and veterinary infectious pathology.  1, record 11, English, - unconventional%20transmissible%20agent

Record 11,  Textual support,  French

Record number: 11, Textual support number: 1 CONT

Sur le plan biochimique, l'agent infectieux des ESST (encéphalophathie subaigüe spongiforme transmissible) présente une résistance inhabituelle à divers procédés d'inactivation : il résiste beaucoup mieux aux agents dénaturant les acides nucléiques (nucléases, radiations ionisantes, psoralènes) qu'aux procédés dénaturant les protéines, comme les protéases. Ces caractéristiques ne sont pas celles généralement observées avec des virus connus, d'où l'expression «agents transmissibles non conventionnels».  1, record 11, French, - agent%20transmissible%20non%20conventionnel

Record 11,  Textual support,  Spanish

Record 12,  Textual support,  English

Record number: 12, Textual support number: 1 DEF

The smallest known infectious particle, a tiny virus-like entity highly resistant to inactivation, composed entirely of a hydrophobic protein and containing no DNA or NRA.  1, record 12, English, - proteinaceous%20infectious%20particle

Record number: 12, Textual support number: 1 CONT

Its molecular weight is 50. 000 or less. Isolated in 1983 from brain tissue of sheep with scrapie, prions are postulated to be the agents of scrapie and possibly of certain slow central nervous system infections of man which resemble scrapie, especially kuru and Creutzfeldt-Jakob disease. It is not known whether prions can reproduce themselves.  1, record 12, English, - proteinaceous%20infectious%20particle

Record 12,  Textual support,  French

Record number: 12, Textual support number: 1 DEF

Constituant principal d'une particule protéique infectieuse jouant un rôle essentiel dans la genèse de certaines maladies et ne possédant ni ARN ou ADN.  3, record 12, French, - prion

Record number: 12, Textual support number: 1 CONT

Baptisé «prion» (anagramme anglo-saxon abrégé de «particule protéique infectieuse»), le microorganisme «non conventionnel» devenait ainsi, soudainement, un véritable hérétique face au dogme sacro-saint de la biologie moléculaire, pour lequel aucune forme de vie reproductible n'est concevable sans acides nucléiques.  4, record 12, French, - prion

Record number: 12, Textual support number: 1 OBS

Comme maladie à prions, on reconnaît la tremblante du mouton ou scrapie, et chez l'humain deux maladies rares, le kuru et la maladie de Creutzfeldt-Jakob.  5, record 12, French, - prion

Record 12,  Textual support,  Spanish

Record number: 12, Textual support number: 1 OBS

De acuerdo con la hipótesis de Stanley B. Prusiner según la cual algunas proteínas pueden comportarse como agentes infecciosos.  2, record 12, Spanish, - pri%C3%B3n

Record 13,  Textual support,  English

Record number: 13, Textual support number: 1 OBS

CJD :Creutzfeldt-Jakob Disease.  2, record 13, English, - CJD%20Surveillance%20Study

Record 13,  Textual support,  French

Record number: 13, Textual support number: 1 OBS

Étude réalisée par le Laboratoire de lutte contre la maladie de Santé Canada.  1, record 13, French, - %C3%89tude%20de%20surveillance%20de%20la%20MCJ

Record number: 13, Textual support number: 2 OBS

MCJ : maladie Creutzfeldt-Jakob.  2, record 13, French, - %C3%89tude%20de%20surveillance%20de%20la%20MCJ

Record 13,  Textual support,  Spanish

Record 14,  Textual support,  English

Record number: 14, Textual support number: 1 CONT

Kuru is a slow-virus disease, once prevalent in New Guinea and rarely seen now. Kuru causes neurodegenerative changes similar to another slow virus disease, Creutzfeldt-Jakob disease, whose distribution is worldwide. In addition, similar slow-virus diseases appear in sheep as scrapie, mink as mink encephalopathy, and in cows as bovine spongiform encephalopathy(BSE). Creutzfeldt-Jakob Disease has been found with increased incidence in North Africa where sheepbrains and eyes are a common part of the diet.  1, record 14, English, - slow%20virus%20disease

Record 14,  Textual support,  French

Record number: 14, Textual support number: 1 CONT

Maladie du système nerveux qui se caractérise par la longueur de l'incubation, le développement lent et irréversible des symptômes conduisant à la mort, des lésions localisées au système nerveux et la possibilité de transmission.  1, record 14, French, - maladie%20%C3%A0%20virus%20lent

Record 14,  Textual support,  Spanish

Record 15,  Textual support,  English

Record number: 15, Textual support number: 1 CONT

Prions mediate the pathogenesis of certain neurodegenerative diseases, including bovine spongiform encephalopathy of cattle and Creutzfeldt-Jakob disease of humans. The prion particle consists mainly-if not entirely-of PrPSc, a posttranslationally modified isoform of the cellular host-encoded prion protein(PrPc). It has been suggested that additional cellular factors might be involved in the physiological function of PrPc and in the propagation of PrPSc.  1, record 15, English, - cellular%20host%2Dencoded%20prion%20protein

Record 15,  Textual support,  French

Record number: 15, Textual support number: 1 CONT

Rappelons brièvement les connaissances acquises sur ces deux isoformes de la PRP (protéine du prion). On l'a vu, la protéine normale présente chez l'individu sain est facilement dégradée par les protéases. Elle a été appelée PrPc (pour cellulaire) ou PrPsen (pour sensible aux protéases), afin de distinguer la forme anormale appelée PrPsc dans de la cas de la tremblante (sc pour scrapie) ou PrPes pour résistante.  1, record 15, French, - PrP%20cellulaire

Record number: 15, Textual support number: 1 OBS

Protéine du prion présent chez l'individu sain.  3, record 15, French, - PrP%20cellulaire

Record 15,  Textual support,  Spanish

Record 16,  Textual support,  French

Record 16,  Textual support,  Spanish

Record 17,  Textual support,  English

Record 17,  Textual support,  French

Record number: 17, Textual support number: 1 OBS

Nouveau système de surveillance mis sur pied au Canada.  1, record 17, French, - Syst%C3%A8me%20de%20surveillance%20de%20la%20maladie%20de%20Creutzfeldt%2DJakob

Record 17,  Textual support,  Spanish