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Summary

Creutzfeldt-Jakob disease (CJD) is a rare, degenerative brain disorder. Symptoms usually start around age 60. Memory problems, behavior changes, vision problems, and poor muscle coordination progress quickly to dementia, coma, and death. Most patients die within a year.

The three main categories of CJD are :

• Sporadic CJD, which occurs for no known reason
• Hereditary CJD, which runs in families
• Acquired CJD, which occurs from contact with infected tissue, usually during a medical procedure

Cattle can get a disease related to CJD called bovine spongiform encephalopathy (BSE) or "mad cow disease." There is concern that people can get a variant of CJD from eating beef from an infected animal, but there is no direct proof to support this.

NIH: National Institute of Neurological Disorders and Stroke

Start Here

• Creutzfeldt-Jakob Disease (Alzheimer's Association)
• Creutzfeldt-Jakob Disease (National Institute of Neurological Disorders and Stroke)
• Transmissible Spongiform Encephalopathies (Prion Diseases) (National Institute of Neurological Disorders and Stroke)

Diagnosis and Tests

• Diagnosing Sporadic Creutzfeldt-Jakob Disease: Accuracy of CSF 14-3-3 Protein Test of the Spinal Fluid (American Academy of Neurology) - PDF

Related Issues

• About Variant Creutzfeldt-Jakob Disease (vCJD) (Centers for Disease Control and Prevention)
• Bovine Spongiform Encephalopathy (BSE), Also Called Mad Cow Disease (Centers for Disease Control and Prevention)
• Information for People Treated with Pituitary Human Growth Hormone (National Institute of Diabetes and Digestive and Kidney Diseases)
• Kuru (National Institute of Neurological Disorders and Stroke)

Specifics

• Frequently Asked Questions on BSE (Bovine Spongiform Encephalopathy or Mad Cow Disease) (Department of Agriculture) - PDF

Genetics

• Prion disease: MedlinePlus Genetics (National Library of Medicine)

Statistics and Research

• Prion Diseases (National Institute of Allergy and Infectious Diseases)

Clinical Trials

• ClinicalTrials.gov: Creutzfeldt-Jakob Syndrome (National Institutes of Health)
• ClinicalTrials.gov: Prion Diseases (National Institutes of Health)

Journal Articles References and abstracts from MEDLINE/PubMed (National Library of Medicine)

• Article: Characterization of variably protease-sensitive prionopathy by capillary electrophoresis.
• Article: Sodium hypochlorite inactivation of human CJD prions.
• Article: Abnormal synaptic architecture in iPSC-derived neurons from a multi-generational family with...
• Creutzfeldt-Jakob Disease -- see more articles

Find an Expert

• Centers for Disease Control and Prevention Also in Spanish
• Food and Drug Administration
• National Institute of Neurological Disorders and Stroke Also in Spanish

Patient Handouts

• Creutzfeldt-Jakob disease (Medical Encyclopedia) Also in Spanish