Creutzfeldt-Jakob disease familial

Creutzfeldt-Jakob disease (CJD) New variant CJD Gerstmann-Straussler-Scheinker disease Fatal familial insomnia Kuru Prion protein Extracellular deposits... 

Human prion disease models have also been developed in mice [154,155]. Crossing the species barrier into an experimentally accessible animal system, the prions responsible for Creutzfeldt Jakob disease, new variant CJD, Gerstmann-Straussler-Scheinker disease, and fatal familial insomnia produce a reproducible time-dependent neuronal degeneration leading to death. 

The prion diseases are a closely related group of neuro-degenerative conditions which affect both humans and animals. They have previously been described as the subacute spongiform encephalopathies, slow virus diseases and transmissible dementias, and include scrapie in sheep, bovine spongiform encephalopathy (BSE) in cattle, and the human prion diseases, Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler-Scheinker disease (GSS), fatal familial insomnia (FFI) and kuru. Prion diseases are... 

CNTF ciliary neurotrophic factor fCJD familial Creutzfeldt-Jakob disease... 

Creutzfeldt-Jakob disease (CJD) Alzheimer s disease (AD) Hemodialysis-related amyloidosis Primary systemic amyloidosis Secondary systemic amyloidosis Familial amyloid polyneuropathy I Familial amyloid polyneuropathy III Cerebral amyloid angiopathy Finnish hereditary systemic amyloidosis Type II diabetes Injection-localized amyloidosis Medullary thyroid carcinoma Atrial amyloidosis... 

Gambetti P, Paixlii P, Petersen RB, Chen SG, Lugai esi P (1995) Eatal familial insomnia and familial Creutzfeldt-Jakob disease Clinical, patliological and molecular featui es. Brain Patliol 5 43—51. 

Creutzfeldt-Jakob disease (CJD) the most common prion disease of humans and can have a sporadic, familial or acquired etiology. 

Fatal insomnia Familial (10-15%) Creutzfeldt-Jakob disease 9 cases Spontaneous... 

To date studies have shown that patients who develop Creutzfeldt-Jakob disease do not have a history of a higher rate of transfusion or treatment with blood products. Likewise, those who have received blood from donors who subsequently prove to have Creutzfeldt-Jakob disease do not acquire an increased risk. Furthermore, animal experiments have shown that blood and blood products are very low risk materials in transfer experiments from diseased to healthy animals. AH the same, certain authorities are now considering the exclusion of donors from families with known cases of classic Creutzfeldt-Jakob disease. 

Creutzfeldt-Jakob disease (CJD), Gerstmann-Straussler-Scheinker syndrome, fatal familial insomnia, and kuru are fatal diseases of the central nervous system, known as transmissible subacute spongiform encephalopathies (193). Creutzfeldt-Jakob disease is the most common of these and has an incidence of 1 1000 000 in most countries. About 10% of those involved have a familial disposition, but most cases occur sporadically. There are similar diseases in sheep (scrapie), cattle (bovine spongiform encephalopathy), and other animal species. The cause of the... 

Goldfarb LG, Brown P, McCombie WR et al (1991) Transmissible familial Creutzfeldt-Jakob disease associated with five, seven, and eight extra octapeptide coding repeats in the PRNP gene. Proc Natl Acad Sci USA 88 10926-10930... 

Silvestrini MC, Cardone F, Maras B, Pucci P, Barra D, Brunori M, Pocchiari M (1997) Identification of the prion protein allotypes which accumulate in the brain of sporadic and familial Creutzfeldt-Jakob disease patients. Nat Med 3 521... 

R, Montagna, R, Ghetti, B., Goldfarb, L.G., Gajdusek, D.C., Lugaresi, E., Gambetti, R., and Autilio-Gambetti, L. (1994). Fatal familial insomnia and familial Creutzfeldt-Jakob disease different prion proteins determined by DNA polymorphism. Proc. Natl. Acad. Sci. U.S.A. 91, 2839-2842. 

Owen, F., Boulter, M., Lofthouse, R., Collinge, J., Crow, T.J., Risby, D., Baker, H.F., Ridley, R.M., Hsiao, K., and Rrusiner, S.B. (1989). Insertion in prion protein gene in familial Creutzfeldt-Jakob disease. Lancet 1, 51-52. 

Creutzfeldt-Jakob disease (CJD) A disease of humans characterized by dementia and destruction of brain tissue, first described by the German psychiatrists H. G. Creutzfeldt (1885-1964) and A. M. Jakob (1884-1931). It is now known to be caused by an abnormal prion protein and is transmissible, although there is also an inherited familial form. This rare disease typically affects middle-aged and elderly people and leads to rapid mental deterioration and death. The abnormal prion interferes with the structure... 

Consider rarer dementias if unusual (particularly neurological) symptoms frontotemporal, dementia in Parkinson s disease, Huntingdon s disease (family histoiy, chorea), Creutzfeldt Jakob Disease, AIDs-related encephalopathy, neurosyphilis, progressive supranuclear palsy. 

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