What Is the Difference Between CJD and vCJD?

Reviewed on 7/26/2021

Classic Creutzfeldt-Jakob disease (CJD) is a rare, neurodegenerative brain disorder that is always fatal. Variant Creutzfeldt-Jakob disease (vCJD) is also fatal and believed to be caused by eating contaminated beef products, similar to bovine spongiform encephalopathy (BSE) or “mad cow disease.”
Classic Creutzfeldt-Jakob disease (CJD) is a rare, neurodegenerative brain disorder that is always fatal. Variant Creutzfeldt-Jakob disease (vCJD) is also fatal and believed to be caused by eating contaminated beef products, similar to bovine spongiform encephalopathy (BSE) or “mad cow disease.”

Classic Creutzfeldt-Jakob disease (CJD) is a rare, neurodegenerative brain disorder that is a type of human prion disease.

The condition progresses quickly and is always fatal, usually within one year of the onset of the illness.

Variant Creutzfeldt-Jakob disease (vCJD) is also a prion disease that is fatal and is thought to be acquired from eating contaminated beef products. It is related to bovine spongiform encephalopathy (BSE), also called “mad cow disease.”

The differences between CJD and vCJD include those listed in the table below.

Differences Between CJD and vCJD
Disease Characteristic

Symptom Comparison

Different initial symptoms

  • Classic CJD symptoms are early neurologic signs and signs of dementia
  • vCJD symptoms are prominent psychiatric and behavioral symptoms, painful sensory distortions, and neurologic signs are delayed
Average duration of illness
  • Classic CJD: 4-5 months
  • vCJD: 13-14 months
Life expectancy
  • Average age at death of vCJD patients is 28 years (vCJD typically occurs in younger patients)
  • Average age at death of CJD patients is 68 years
Causes
  • CJD is caused by a genetic mutation
  • vCJD is caused by consuming contaminated beef products or rarely, by blood transfusion or rarely, by blood transfusion

What Are Symptoms of CJD and vCJD?

Classic Creutzfeldt-Jakob disease (CJD) is characterized by rapidly progressive neurologic signs and dementia. Initial symptoms may include: 

  • Problems with muscle coordination
  • Personality changes (including impaired memory, judgment, and thinking)
  • Impaired vision
  • Insomnia
  • Depression
  • Unusual sensations

In later stages, symptoms of classic CJD include: 

  • Severe mental impairment 
  • Involuntary muscle jerks (myoclonus)
  • Blindness
  • Loss of ability to move and speak
  • Coma
  • Pneumonia and other infections 
  • Death

Symptoms of variant Creutzfeldt-Jakob disease (vCJD) include: 

  • Psychiatric symptoms at illness onset 
  • Persistent painful sensory symptoms 
  • Dementia, and development 4 or more months after illness onset of at least two of the following five neurologic signs: 
    • Poor coordination
    • Involuntary muscle jerks (myoclonus)
    • Brief, involuntary, irregular muscle movements (chorea)
    • Overactive reflex response 
    • Visual signs

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What Causes CJD and vCJD?

Both classic Creutzfeldt-Jakob disease (CJD) and variant Creutzfeldt-Jakob disease (vCJD) are caused by a buildup of abnormal prion proteins, which attach to other brain cell proteins and bend them, eventually killing clusters of brain cells. Prion infections also cause small holes to develop in the brain, which makes it sponge-like. The damage to the brain tissue results in the symptoms of classic CJD and vCJD, and eventually leads to death.

Classic CJD is caused by a genetic mutation in the gene for the body’s normal prion protein. In the genetic version of CJD, a mutation is passed from parent to child. In the sporadic form of CJD, infectious prions are believed to be made by an error of the cell mechanism that makes proteins and controls their quality.  

vCJD is caused by consuming contaminated beef products or rarely, by blood transfusion.

How Are CJD and vCJD Diagnosed?

Tests used to help diagnose classic Creutzfeldt-Jakob disease (CJD) and variant Creutzfeldt-Jakob disease (vCJD) include: 

However, brain biopsy or autopsy is the only way to confirm a diagnosis of classic CJD or vCJD.  

What Is the Treatment for CJD and vCJD?

There is currently no treatment to cure or manage prion diseases such as classic Creutzfeldt-Jakob disease (CJD) and variant Creutzfeldt-Jakob disease (vCJD). The goal of treatment is to relieve symptoms and make the person as comfortable as possible. 

Treatments for both classic CJD and vCJD may include: 

  • Opioids for pain 
  • Clonazepam and sodium valproate for myoclonus
  • Intravenous fluids
  • Artificial feeding 

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Reviewed on 7/26/2021
References
https://www.cdc.gov/prions/vcjd/index.html

https://www.cdc.gov/prions/cjd/index.html

https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Creutzfeldt-Jakob-Disease-Fact-Sheet

https://www.healthlinkbc.ca/healthlinkbc-files/variant-creutzfeldt-jakob-disease