CJD Creutzfeldt-Jakob

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... 

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... 

Bovine spongiform encephalopathy (BSE or mad cow disease) is a progressive neurological degenerative disease in cattle. It is caused by a mutated protein called a prion. BSE was first reported in the United Kingdom in 1986. Creutzfeldt-Jakob disease (CJD) is a rare disease that occurs in humans. Evidence to date indicates it is possible for humans to acquire CJD after consuming BSE-contaminated cattle products. 

With the background of the mad cow crisis in Europe, questions relating to the prion diseases have attracted intensive interest. It is now widely accepted that prion diseases, such as Creutzfeldt-Jakob disease (CJd) in humans and bovine spongiform encephalopathy (BSE) are caused by a conformational change of the prion protein (PrP) from a normally folded cellular form, PrP ", to an alternate, aggregation-prone, pathogenic scrapie form,... 

Albumin (human) Darbepoetin formulated with albumin carries an extremely remote risk for transmission of viral diseases. A theoretical risk for transmission of Creutzfeldt-Jakob disease (CJD) also is considered extremely remote. No cases of transmission of viral disease or CJD have ever been identified for albumin. Pregnancy Category C. 

The use of hGH extracted from the pituitaries of deceased human donors came to an abrupt end in 1985, when a link between treatment and Creutzfeldt-Jakob disease (CJD, a rare, but fatal, neurological disorder) was discovered. In this year, a young man who had received hGH therapy some 15 years previously died from CJD, which, investigators concluded, he had contracted from infected pituitary extract (CJD appears to be caused by a prion). At least an additional 12 CJD cases suspected of being caused in the same way have subsequently been documented. Fortunately, several recombinant hGH (rhGH) preparations were coming onstream at that time (Table 8.8), and now all hGH preparations used clinically are derived from recombinant sources. Currently, in excess of 20 000 people are in receipt of rhGH therapy. 

Prion diseases have attracted immense attention over the past decade, prompted, in part, by the outbreak of mad cow disease in the United Kingdom. The most common prion disease is sporadic Creutzfeldt-Jakob disease (CJD). Clinically, CJD is characterized by a rapidly progressive dementia accompanied variably by early-onset seizures, insomnia, disordered movements, and psychiatric disturbances the disease is uniformly fatal. Histochemically, the principal pathological feature of prion disease is the abnormal accumulation of an amyloid-like material composed of prion protein (PrP), which is encoded by a single gene on the short arm of chromosome 20. 

Up through 1980, people thought that all infectious diseases were caused by microbes of some sort. They knew about diseases caused by viruses, bacteria, protozoa, and fungi. There were some strange diseases, however, for which no one had isolated and cultured the pathogen. Creutzfeldt Jakob Disease (CJD) in humans, scrapie in sheep, and transmissible encephalopathy in mink (TME) all involved a slow, gradual loss of mental function and eventual death. The brains of the victims all showed unusual plaques of amyloid protein surrounded by spongelike tissue. 

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