Creutzfeldt-Jakob Disease, New Variant Creutzfeldt-Jakob Disease and Bovine Spongiform Encephalopathy – An Update

Bovine spongiform encephalopathy and new variant Creutzfeldt-Jakob disease.

Bovine spongiform encephalopathy (BSE) and Creutzfeldt-Jakob Disease (CJD) belong to a group of degenerative neurological disorders collectively known as the transmissible spongiform encephalopathies (TSEs). The group also includes scrapie of sheep and goats, kuru of humans, chronic wasting disease of mule deer and elk and transmissible encephalopathy of mink. These fatal diseases cause behavio...

Bovine Spongiform Encephalopathy, Creutzfeldt-Jakob Disease and Immunizations

BSE (bovine spongiform encephalopathy) is a progressive neurological disorder of cattle, and has been called “mad cow disease.” Its symptoms are similar to “scrapie,” a brain disease that occurs in sheep. Cattle affected by BSE experience progressive degeneration of the nervous system. Affected animals may display changes in temperament, such as nervousness or aggression, abnormal posture, inco...

Bovine Spongiform Encephalopathy and Its Relationship to Creutzfeldt-jakob Disease

Variant Creutzfeldt-Jakob disease: an update.

Variant Creutzfeldt-Jakob disease (vCJD) is a novel human prion disease caused by the bovine spongiform encephalopathy agent. Most cases have occurred in the UK, with smaller numbers in 11 other countries. All definite vCJD cases have occurred in methionine homozygotes at codon 129 in the prion protein gene. Following oral infection, the vCJD agent appears to replicate in lymphoid tissues durin...

Genetic Creutzfeldt-Jakob disease mimicking variant Creutzfeldt-Jakob disease.

The ‘‘pulvinar sign’’ on MRI brain scan is defined as hyperintensity of the posterior thalamus relative to the signal intensity of the anterior putamen. In the appropriate clinical context, the pulvinar sign and hyperintensity in the dorsomedial thalamic nuclei (the hockey stick sign) are sensitive and specific features of variant Creutzfeldt– Jakob disease (vCJD). Variant CJD is an acquired fo...