Creutzfeldt-Jakob disease
Creutzfeldt-Jakob disease: Excerpt from Professional Guide to Diseases (Eighth Edition)
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. CJD is always fatal. A new variant of CJD (vCJD) emerged in Europe in 1996. (See Understanding vCJD.)
Causes and incidence
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.
CJD generally affects adults ages 40 to 65 and occurs in more than 50 countries. Males and females are affected equally. In people younger than age 30, incidence is 5 in 1,000,000,000; in all other age groups, incidence is 1 in 1,000,000.
Signs and symptoms
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. Involuntary movements, such as muscle twitching, trembling, and peculiar body movements, and visual disturbances, appear with disease progression and advancing mental deterioration. Hallucinations are also common. Duration of the typical illness is 4 months.
Diagnosis
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 EEG may 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. Though not diagnostic, presence of the 14-3-3 protein in the spinal fluid is highly suggestive of the disease when it’s accompanied by other characteristic symptoms. Definitive diagnosis usually isn’t obtained until an autopsy is done and brain tissue is examined.
Treatment
There’s no cure for CJD, and its progress can’t be slowed. Palliative care is provided to make the patient comfortable and to ease symptoms. Medications may be needed to control aggressive behaviors. These include sedatives and antipsychotics.
The need to provide a safe environment, control aggressive or agitated behavior, and meet 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.
Behavior modification may be helpful, in some cases, for controlling unacceptable or dangerous behaviors. 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.
Special considerations
❑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 patient and his family to discuss and complete advance directives.
❑To prevent disease transmission, use caution when handling body fluids and other materials from patients suspected of having CJD.
Pictures
Book Source Details
- Book Title: Professional Guide to Diseases (Eighth Edition)
- Author(s): Springhouse
- Year of Publication: 2005
- Copyright Details: Professional Guide to Diseases (Eighth Edition), Copyright © 2005 Lippincott Williams & Wilkins.
More About Adrenoleukodystrophy
More Medical Textbooks Online about Adrenoleukodystrophy
Review other book chapters online related to Adrenoleukodystrophy:
Medical Books Excerpts
- SPASTICITY
- "Algorithmic Diagnosis of Symptoms and Signs" (2003)
- [ read ]
- Skin, bronze
- "Professional Guide to Signs & Symptoms (Fifth Edition)" (2006)
- [ read ]
Copyright notice for book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.
» Next page: Encephalitis (Professional Guide to Diseases (Eighth Edition))
Rate This Website
What do you think about the features of this website?
Take our user survey and have your say:
Website User Survey
Medical Tools & Articles:
Next articles:
Tools & Services:
Medical Articles:
Forums & Message Boards
- Ask or answer a question at the Boards:
