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prions

Clinical Features

cjd

Examination of brain tissue reveals florid plaques of agglomerated prions and vacuolization.

http://pathology.mc.duke.edu/neuropath/CNSlecture2/cjd.jpg


IncubationLong incubation periods of up to 30 years
OutcomeSymptoms are progressive and result in fatality
IncubationLong incubation periods of up to 30 years
PathologyPrions cause spongiform degeneration, a process of vacuolization in which the brain deteriorates and looks spongy
SymptomsBehavorial effects include ataxia, peripheral sensory changes and dementia
DiagnosisDiseases are invariably fatal, and definite diagnosis can usually only be made at autopsy


Human Prion DiseasesAnimal Prion Diseases
Creutzfeldt-Jakob Disease (CJD)
Variant Creutzfeldt-Jakob Disease (vCJD)
Gerstmann-Straussler-Scheinker Syndrome
Fatal Familial Insomnia
Kuru		 Bovine Spongiform Encephalopathy (BSE)
Scrapie
Chronic Wasting Disease (CWD)
Transmissible mink encephalopathy
Feline spongiform encephalopathy
Ungulate spongiform encephalopathy

    Human TSEs occur in sporadic, familial, and acquired forms. The most common form, sporadic Creutzfeldt-Jakob disease (CJD), has a worldwide death rate of about 1 case per million people each year and is responsible for about 85% of all CJD cases worldwide (WHO). Familial Creutzfeldt-Jakob disease (fCJD) is inherited as a result of genetic mutations and counts for 10-15% of the cases worldwide (WHO). The remaining cases are iatrogenic (accidental transmission through contaminated surgical equipment, as a result of cornea or dura mater transplants, or due to the administration of human growth hormones) and variant Creutzfeldt-Jakob disease (WHO).