Creutzfeldt-Jakob disease

GC: n

S: NIH – (last access: 21 August 2014); WHO – (last access: 21 August 2014).

N: 1. Creutzfeld-Jakob disease (CJD) is a transmissible spongiform encephalopathy. Until the 1990s, three forms of CJD were recognized: sporadic disease, which accounted for the majority of cases and was of unknown origin; familial cases, associated with a gene mutation; and iatrogenic cases, caused by accidental transmission of the causative agent via contaminated instruments or certain transplants. Most cases were seen in people over 50 years of age, and were characterized by personality changes and progressive dementia. Death usually occurred within one year.
2. In the 1990s, a variant of CJD was recognized (vCJD), which was strongly linked to exposure – probably through food – to a disease of cattle, bovine spongiform encephalopathy. vCJD tends to affect younger patients than classical CJD, and to have a longer duration. Early symptoms include depression or, occasionally, a schizophrenia-like psychosis. As the disease progresses, patients develop more and more neurological signs, including unsteadiness, difficulty walking and involuntary movements.
3. Creutzfeldt-Jakob disease (CJD), rare fatal degenerative disease of the central nervous system. CJD occurs throughout the world at an incidence of one in every one million people. Among certain populations, such as Libyan Jews, rates are somewhat higher.
4. The disease was first described in the 1920s by the German neurologists Hans Gerhard Creutzfeldt and Alfons Maria Jakob. CJD is similar to other neurodegenerative diseases such as kuru, a human disorder, and scrapie, which occurs in sheep and goats. All three diseases are types of transmissible spongiform encephalopathies, so called because of the characteristic spongelike pattern of neuronal destruction that leaves brain tissue filled with holes.

S: 1 & 2. WHO – (last access: 21 August 2014). 3 & 4. EncBrit – (last access: 21 August 2014).

SYN: CJD, Creutzfeldt-Jakob syndrome, Jakob-Creutzfeldt disease, transmissible dementia.

S: GDT – (last access: 21 August 2014)

CR: bovine spongiform encephalopathy, infectious disease, transmissible spongiform encephalopathy, dementia.