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Creutzfeldt-Jakob Disease (CJD)

Overview

Creutzfeldt-Jakob disease (CJD) is a life-threatening and rare neurodegenerative brain disorder. It can be sporadic, familial (inherited), iatrogenic (acquired), or variant (infection). The symptoms progress rapidly, causing deterioration in sensorium and encephalopathy. Sadly, most patients do not survive beyond 6–12 months after symptoms appear.

CJD can occur in different forms:

  • Sporadic CJD (sCJD): Most common form, develops without known cause.
  • Familial CJD: Caused by inherited genetic changes.
  • Iatrogenic CJD: Acquired through medical procedures involving contaminated tissue.
  • Variant CJD (vCJD): Caused by eating beef contaminated with abnormal prion proteins.

The symptoms of CJD progress rapidly and may include:

  • Rapidly progressive dementia (loss of memory, thinking ability, and judgment)
  • Behaviour changes (personality change, anxiety, depression)
  • Hallucinations (seeing or hearing things that are not there)
  • Jerky movements/ myoclonus
  • Balance and coordination dysfunction
  • Speech impairment

CJD is caused by an abnormal form of a protein called a prion. Prions misfold and accumulate in the brain, damaging brain cells and causing the disease.

Doctors use a combination of tests to diagnose CJD:

  • EEG (electroencephalogram): Measures brain activity; in CJD it shows repeated abnormal discharges.
  • CSF examination: For detecting biomarkers like 14-3-3, RT-QuIC for sporadic CJD
  • MRI: To determine characteristic patterns such as “cortical ribboning” and “inverse hockey stick” sign
  • Brain biopsy: Rarely performed, but can confirm the diagnosis. 

There is no cure for CJD. Treatment focuses on easing symptoms and improving comfort:

  • Medicines: For symptomatic control of seizures and myoclonus
  • Supportive care: For pain relief and nutrition
  • Palliative care: To support quality of life and reduce suffering

Consult a doctor if you or someone you know develops:

  • Rapid memory loss or confusion
  • Sudden changes in behaviour or hallucinations
  • Loss of coordination or balance
  • Jerky movements or muscle stiffness

There is currently no way to prevent sporadic or inherited CJD. However:

  • Medical precautions, such as sterilising surgical instruments, help prevent iatrogenic CJD.
  • Avoiding contaminated beef products reduces the risk of variant CJD.

For diagnosis and management of CJD, consult a Neurologist (specialist in brain and nerve disorders).

Disclaimer:

The information in this Health Library is for educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always consult a qualified healthcare professional with any questions about a medical condition or before starting any treatment. Use of this site and its content does not establish a doctor–patient relationship. In case of a medical emergency, call your local emergency number or visit the nearest emergency facility immediately.