Transmissible spongiform encephalopathy; vCJD; CJD; Jacob-Creutzfeldt disease
There is no known cure. Interleukins and other medications may be use in an attempt to slow the progression of the disease. Custodial care may be required early in the course of the disease. Medications may be needed to control aggressive behaviors.
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.
As with other illnesses that cause dementia, 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.
The outcome is usually very poor. Within 6 months or less of the onset of symptoms, the person will become incapable of self-care.
The disorder is fatal in a short time, usually within 8 months, but a few people survive as long as 1 or 2 years after diagnosis. The cause of death is usually infection, heart failure, or respiratory failure.
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.
Zeidler M. Prion diseases. In: Goldman L, Ausiello D, eds. Cecil Medicine. 23rd ed. Philadelphia, Pa: Saunders Elsevier. 2007: chap 442.
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