Condition 101 About Creutzfeldt-Jakob Disease

What is the definition of Creutzfeldt-Jakob Disease?

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

What are the alternative names for Creutzfeldt-Jakob Disease?

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

What are the causes for Creutzfeldt-Jakob Disease?

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:

  • Sporadic CJD makes up most cases. It occurs for no known reason. The average age at which it starts is 65.
  • Familial CJD occurs when a person inherits the abnormal prion from a parent (this form of CJD is rare).
  • Acquired CJD includes variant CJD (vCJD), the form related to mad cow disease. Iatrogenic CJD is also an acquired form of the disease. Iatrogenic CJD is sometimes passed through a blood product transfusion, transplant, or contaminated surgical instruments.

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:

  • Chronic wasting disease (found in deer)
  • Kuru (affected mostly women in New Guinea who ate the brains of dead relatives as part of a funeral ritual)
  • Scrapie (found in sheep)
  • Other very rare inherited human diseases, such as Gerstmann-Straussler-Scheinker disease and fatal familial insomnia

What are the symptoms for Creutzfeldt-Jakob Disease?

CJD symptoms may include any of the following:

  • Dementia that gets worse quickly over a few weeks or months
  • Blurred vision (sometimes)
  • Changes in gait (walking)
  • Confusion, disorientation
  • Hallucinations (seeing or hearing things that aren't there)
  • Lack of coordination (for example, stumbling and falling)
  • Muscle stiffness, twitching
  • Feeling nervous, jumpy
  • Personality changes
  • Sleepiness
  • Sudden jerky movements or seizures
  • Trouble speaking

What are the current treatments for Creutzfeldt-Jakob Disease?

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.

What is the outlook (prognosis) for Creutzfeldt-Jakob Disease?

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:

  • Infection with the disease
  • Severe malnutrition
  • Dementia in some cases
  • Loss of ability to interact with others
  • Loss of ability to function or care for oneself
  • Death

When should I contact a medical professional for Creutzfeldt-Jakob Disease?

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.

How do I prevent Creutzfeldt-Jakob Disease?

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.

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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.

Top Global Doctors For Creutzfeldt-Jakob Disease

FL
Elite
Franc Llorens
Barcelona, CT, ES
SM
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Simon H. Mead
London, ENG, GB
YI
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Yasushi Iwasaki
Nagakute, JP
IZ
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Inga Zerr
Goettingen, NI, DE
GK
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Gabor G. Kovacs
Vienna, AT

Latest Advances On Creutzfeldt-Jakob Disease

  • Condition: Chronic Kidney Disease (CKD)
  • Journal: International journal of molecular sciences
  • Treatment Used: Pioglitazone and Human Mesenchymal Stem Cells (MSCs)
  • Number of Patients: 0
  • Published —
This study evaluated the protective effect of pioglitazone on mesenchymal stem cells (MSCs) isolated from patients with chronic kidney disease (CKD-MSCs) against chronic kidney disease induced endoplasmic reticulum stress.
  • Condition: Colorectal cancer
  • Journal: Journal of pineal research
  • Treatment Used: Anticancer drug and melatonin
  • Number of Patients: 0
  • Published —
The study researched the effects of melatonin and 5-fluorouracil in patients with colorectal cancer.

Clinical Trials For Creutzfeldt-Jakob Disease

Clinical Trial
  • Status: Recruiting
  • Phase: Phase 4
  • Intervention Type: Device
  • Participants: 250
  • Start Date: March 2016
Study of Nasal Brushing Collected OLFActory MUcosa Samples in the Diagnosis of Human Encephalopathies