This is a group of rare diseases in which a transmissible agent known as 'prion protein' accumulates in the brain. This causes dementia and neurological symptoms including unsteadiness and jerky movements. Different prion diseases occur in humans and animals. One of these, Creutzfeldt-Jakob disease (CJD), has been identified for some time in a small number of humans. More recently, a new form of CJD, known as variant CJD, has been identified.

An overview of human prion diseases
Prion diseases are transmissible, progressive and invariably fatal neurodegenerative conditions associated with misfolding and aggregation of a host-encoded cellular prion protein, PrP(C). They have o
http://www.virologyj.com/content/8/1/559
Creutzfeldt-Jakob Disease, from the Alzheimer's Society of Canada
Creutzfeldt-Jakob Disease Introduction Dementia is a syndrome consisting of a number of symptoms that include loss of memory, judgment and reasoning, and changes in mood, behaviour and communicati
http://www.alzheimer.ca/english/disease/dementias-...
The genetics of prion diseases
Prion diseases are a rare group of fatal neurodegenerative disorders of humans and animals that manifest primarily as progressive dementia and ataxia. Unique to these diseases is the prion, a misfolde
http://www.nature.com/gim/journal/v12/n4/full/gim2...
Updated clinical diagnostic criteria for sporadic Creutzfeldt-Jakob disease
Several molecular subtypes of sporadic Creutzfeldt–Jakob disease have been identified and electroencephalogram and cerebrospinal fluid biomarkers have been reported to support clinical diagnosis but
http://brain.oxfordjournals.org/content/132/10/265...
What is Creutzfeldt-Jakob disease (CJD)? from the Alzheimer's Society, U.K.
What is Creutzfeldt-Jakob disease (CJD)? Creutzfeldt-Jakob disease (CJD) is the best known of a group of diseases called prion disease, which affect a form of protein found in the central nervous s
http://www.alzheimers.org.uk/site/scripts/document...