Creutzfeldt-Jakob disease

Definition

Creutzfeldt-Jakob disease (CJD) is a rare, fatal neurodegenerative disorder caused by prions. It leads to rapidly progressive dementia and other neurological symptoms.

5 Must Know Facts For Your Next Test

1. CJD is caused by misfolded prion proteins that induce other normal proteins to misfold.
2. The disease can be sporadic, hereditary, or acquired through exposure to infected tissue.
3. Symptoms include rapid mental deterioration, muscle stiffness, and involuntary movements.
4. There is currently no cure for CJD, and it usually leads to death within a year of onset.
5. Diagnosis typically involves EEG, MRI, cerebrospinal fluid analysis, and sometimes brain biopsy.

Review Questions

• What causes Creutzfeldt-Jakob disease?
• What are the main types of Creutzfeldt-Jakob disease?
• How is Creutzfeldt-Jakob disease diagnosed?

Related terms

Prions: Infectious agents composed of protein that cause neurodegenerative diseases by inducing abnormal folding of specific normal cellular proteins.

Kuru: A prion disease similar to CJD found in the Fore people of Papua New Guinea, transmitted through ritualistic cannibalism.

Bovine Spongiform Encephalopathy (BSE): Also known as 'mad cow disease,' BSE is a prion disease affecting cattle that can be transmitted to humans as variant CJD (vCJD).