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Creutzfeldt-Jakob disease

Transmissible spongiform encephalopathy; vCJD; CJD; Jacob-Creutzfeldt disease

Creutzfeldt-Jakob disease (CJD) is a form of brain damage that leads to a rapid decrease in movement and loss of mental function.

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Creutzfeldt-Jakob disease
Central nervous system and peripheral nervous system

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Causes

CJD is caused by a protein called a prion. A prion causes normal proteins to fold abnormally. This affects other proteins' ability to function.

CJD is very rare. There are several types. The classic types of CJD are:

Variant CJD is caused by eating infected meat. The infection that causes the disease in cows is believed to be the same one that causes vCJD in humans.

Variant CJD causes less than 1 percent of all CJD cases. It tends to affect younger people. Fewer than 200 people worldwide have had this disease. Almost all cases occurred in England and France.

CJD may be related to several other diseases caused by prions, including:

Symptoms

CJD symptoms may include any of the following:

Exams and Tests

Early in the disease, a nervous system and mental examination will show memory and thinking problems. Later in the disease, a motor system examination (an exam to test muscle reflexes, strength, coordination, and other physical functions) may show:

There is a loss of coordination and changes in the cerebellum. This is the area of the brain that controls coordination.

An eye exam shows areas of blindness that the person may not notice.

Tests used to diagnose this condition may include:

The disease can only be confirmed with a brain biopsy or autopsy. Today, it is very rare for a brain biopsy to be done to look for this disease.

Treatment

There is no known cure for this condition. Different medicines have been tried to slow the disease. These include antibiotics, drugs for epilepsy, blood thinners, antidepressants, and interferon. But none works well.

The goal of treatment is to provide a safe environment, control aggressive or agitated behavior, and meet the person's needs. This may require monitoring and assistance in the home or in a care facility. Family counseling may help the family cope with the changes needed for home care.

People with this condition may need help controlling unacceptable or dangerous behaviors. This involves rewarding positive behaviors and ignoring negative behaviors (when it is safe). They may also need help getting oriented to their surroundings. Sometimes, medicines are needed to help control aggression.

Persons with CJD and their family may need to seek legal advice early in the course of the disorder. Advance directive, power of attorney, and other legal actions can make it easier to make decisions about the care of the person with CJD.

Outlook (Prognosis)

The outcome of CJD is very poor. People with sporadic CJD are unable to care for themselves within 6 months or less after symptoms begin.

The disorder is fatal in a short time, usually within 8 months. People who have variant CJD get worse more slowly, but the condition is still fatal. A few people survive for as long as 1 or 2 years. The cause of death is usually infection, heart failure, or respiratory failure.

The course of CJD is:

When to Contact a Medical Professional

CJD is not a medical emergency. However, getting diagnosed and treated early may make the symptoms easier to control, give patients time to make advance directives and prepare for the end of life, and give families extra time to come to terms with the condition.

Prevention

Medical equipment that may be contaminated should be removed from service and disposed of. People known to have CJD should not donate a cornea or other body tissue.

Most countries now have strict guidelines for managing infected cows to avoid transmitting CJD to humans.

Related Information

Protein in diet
Growth hormone test
Kuru
Encephalitis

References

Bosque PJ, Tyler KL. Prions and prion disease of the central nervous system (transmissible neurodegenerative diseases). In: Bennett JE, Dolin R, Blaser MJ, eds. Mandell, Douglas, and Bennett's Principles and Practice of Infectious Diseases. 9th ed. Philadelphia, PA: Elsevier; 2020:chap 179.

Geschwind MD. Prion diseases. In: Daroff RB, Jankovic J, Mazziotta JC, Pomeroy SL, eds. Bradley's Neurology in Clinical Practice. 7th ed. Philadelphia, PA: Elsevier; 2016:chap 94.

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Review Date: 6/23/2019  

Reviewed By: Alireza Minagar, MD, MBA, Professor, Department of Neurology, LSU Health Sciences Center, Shreveport, LA. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

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