Creutzfeldt-Jakob disease

Alternative Names

Treatment

There is no known cure. Custodial care may be required early in the course of the disease. Medications may be needed to control aggressive behaviors. These include sedatives, antipsychotics, and others.

Provding a safe environment, controlling aggressive or agitated behavior, and meeting physiologic needs may require monitoring and assistance in the home or in an institutionalized setting. Family counseling may help in coping with the changes required for home care.

Visiting nurses or aides, volunteer services, homemakers, adult protective services, and other community resources may be helpful in caring for the person with CJD.

Behavior modification may be helpful, in some cases, for controlling unacceptable or dangerous behaviors. This consists of rewarding appropriate or positive behaviors and ignoring inappropriate behaviors (within the bounds of safety). Reality orientation, with repeated reinforcement of environmental and other cues, may help reduce disorientation.

Legal advice may be appropriate early in the course of the disorder to form advance directives, power of attorney, and other legal actions that may make it easier to make ethical decisions regarding the care of an individual with CJD.

Outlook (Prognosis)

The outcome is usually very poor. Complete dementia commonly occurs within 6 months or less of the onset of symptoms, with the person becoming totally incapable of self-care.

The disorder is fatal in a short time, usually within 7 months, but a few people survive as long as 1 or 2 years after diagnosis of the disorder. The cause of death is usually infection, heart failure, or respiratory failure.

Possible Complications

• Infection
• Heart failure
• Respiratory failure
• Loss of ability to function or care for oneself
• Loss of ability to interact with others
• Death

When to Contact a Medical Professional

CJD is not a medical emergency, but early diagnosis and treatment may make the symptoms easier to control, allow patients time to make advance directives, and give families additional time to come to terms with the condition.

References

Llewelyn CA, Hewitt PE, et al. Possible transmission of variant Creutzfeldt-Jakob disease by blood transfusion. Lancet 2004;363:417-421.

Peden AH, Head MW, et al. Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient. Lancet 2004;264:527-529.

Brown P, Will RG, Bradley R, Asher DM, Detwiler L. Bovine Spongiform Encephalopathy and Variant Creutzfeldt-Jakob Disease: Background, Evolution, and Current Concerns. EID. January-February 2001; 7(1):6-16.

Noble J. Textbook of Primary Care Medicine. 3rd ed. St. Louis, Mo: Mosby; 2001:1551-1569.