Creutzfeldt-Jakob disease (CJD) is a rare, rapidly progressive viral disease that attacks the central nervous system, causing dementia and neurologic signs and symptoms, such as myoclonic jerking, ataxia, aphasia, visual disturbances, and paralysis. It generally affects adults ages 40 to 65 and occurs in more than 50 countries. Males and females are affected equally. CJD is always fatal. A new variant of CJD (nvCJD) emerged in Europe in 1996. (See Understanding nvCJD.)
The causative organism is difficult to identify because no foreign ribonucleic acid or deoxyribonucleic acid has been linked to the disease. CJD is believed to be caused by a specific protein called a prion, which lacks nucleic acids, resists proteolytic digestion, and spontaneously aggregates in the brain. Most cases are sporadic; 5% to 15% are familial, with an autosomal dominant pattern of inheritance. Although CJD isn’t transmitted by normal casual contact, human-to-human transmission can occur as a result of certain medical procedures, such as corneal and cadaveric dura mater grafts. Isolated cases are attributed to treatment during childhood with human growth hormone and to improperly decontaminated neurosurgical instruments and brain electrodes.
Early signs and symptoms of mental impairment may include slowness in thinking, difficulty concentrating, impaired judgment, and memory loss. Dementia is progressive and occurs early. With disease progression and mental deterioration, involuntary movements, such as muscle twitching, trembling, and peculiar body movements, and vision disturbances appear. Hallucinations also are common. Duration of the typical illness is 4 months.
CJD must be considered for anyone experiencing signs of progressive dementia. Neurologic examination is the most effective tool in diagnosing CJD. Difficulty with rapid alternating movements and point-to-point movements are typically evident early in the disease.
An electroencephalogram may also be performed to assess the patient for typical changes in brain wave activity. Computed tomography scan, magnetic resonance imaging of the brain, and lumbar puncture may be useful in ruling out other disorders that cause dementia. Definitive diagnosis usually isn’t obtained until an autopsy is done and brain tissue is examined.
There is no cure for CJD, and its progress can’t be slowed. Palliative care is provided to make the patient comfortable and to ease symptoms.
❑ Offer emotional support to the patient and his family. Teach them about the disease, and assist them through the grieving process. Refer the patient and his family to CJD support groups, and encourage participation.
❑ Contact social services and hospice, as appropriate, to assist the family with their needs.
❑ Encourage the family and patient to discuss and complete advance directives.
Clinical tip To prevent disease transmission, use caution when handling body fluids and other materials from patients suspected of having CJD.
Review other book chapters online related to Creutzfeldt-Jakob Disease:
Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
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Title: Handbook of Diseases Authors: Springhouse Publisher: Lippincott Williams & Wilkins Copyright: 2003 ISBN: 1-58255-266-5 
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