CJD; Prion disease; Transmissible spongiform encephalopathy (TSE
Short Description or Definition
Creutzfeldt-Jakob Disease (CJD) is a rare, fatal neurodegenerative disease, which is one of the transmissible spongiform encephalopathies or prion diseases. These conditions are characterized pathologically by neuronal loss, spongiform change, and astrocytic gliosis. Cell loss can be seen microscopically as multiple perforations to the brain tissue creating the characteristic “spongelike” appearance. Prion diseases are caused by infectious agents, which are abnormal self-replicating forms of a normal brain protein, prion protein.
Categorization and Epidemiology
Creutzfeldt-Jakob disease (CJD) may be sporadic (that is develop spontaneously without apparent cause), familial (inherited), or acquired (transmitted by infection).
CJD occurs worldwide with a mean annual incidence of approximately one to two cases per million population (Ladogana et al. 2005). Except for variant and...
References and Readings
- Clare, L. (2007). Neuropsychological rehabilitation and people with dementia. Hove: Psychology Press.Google Scholar
- Creutzfeldt-Jakob Disease Foundation website. (2015). http://www.cjdfoundation.org
- Medical Research Council New Therapies Scrutiny Group for Prion Disease website. (2015). http://www.mrc.ac.uk/PolicyGuidance/PolicyDevelopment/NewTherapiesScrutinyGroupforPrionDisease
- National Creutzfeldt-Jakob Disease Surveillance Unit (NCJDSU) website. (2015). http://www.cjd.ed.ac.uk
- National Prion Clinic website. (2015). http://www.nationalprionclinic.org