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Creutzfeldt-Jakob
Introduction
- What is
Creutzfeldt-Jakob?
Creutzfeldt-Jakob disease (CJD) is a rare,
degenerative, invariably fatal brain disorder. Typically, onset of symptoms
occurs at about age 60. There are three major categories of CJD: sporadic CJD,
hereditary CJD, and acquired CJD. There is currently no single diagnostic test
for CJD. The first concern is to rule out treatable forms of dementia such as
encephalitis or chronic meningitis. The only way to confirm a diagnosis of CJD
is by brain biopsy or autopsy. In a brain biopsy, a neurosurgeon removes a small
piece of tissue from the patient's brain so that is can be examined by a
neurologist. Because a correct diagnosis of CJD does not help the patient, a
brain biopsy is discouraged unless it is need to rule out a treatable disorder.
While CJD can be transmitted to other people, the risk of this happening is
extremely small.
Is
there any treatment?
There is no treatment that can cure or control CJD.
Current treatment is aimed at alleviating symptoms and making the patient as
comfortable as possible. Opiate drugs can help relieve pain, and the drugs
clonazepam and sodium valproate may help relieve involuntary muscle jerks.
What
is the prognosis?
About 90 percent of patients die within 1 year. In the
early stages of disease, patients may have failing memory, behavioral changes,
lack of coordination and visual disturbances. As the illness progresses, mental
deterioration becomes pronounced and involuntary movements, blindness, weakness
of extremities, and coma may occur.
What research
is being done?
The leading scientific theory at this time maintains
that CJD is caused by a type of protein called a prion. The harmless and the
infectious forms of the prion protein are nearly identical, but the infectious
form takes a different folded shape than the normal protein. Researchers are
examining whether the transmissible agent is, in fact, a prion and trying to
discover factors that influence prion infectivity and how the disorder damages
the brain. Using rodent models of the disease and brain tissue from autopsies,
they are also trying to identify factors that influence the susceptibility to
the disease and that govern when in life the disease appears.
Organizations
Alzheimer's Association
919 N. Michigan Avenue
Suite 1100
Chicago IL 60611-1676
info@alz.org
www.alz.org
Tel: 312-335-8700 800-272-3900
Fax: 312-335-1110
CDC/Centers for Disease Control
1600 Clifton Road, NE
Atlanta GA 30333
inquiry@cdc.gov
www.cdc.gov
Tel: 800-311-3435
Creutzfeldt-Jakob (CJD) Foundation Inc.
P.O. Box 611625
No. Miami FL 33261-1625
crjakob@aol.com
Fax: 954-436-7591
National Organization for Rare Disorders (NORD)
P.O. Box 8923
(100 Route 37)
New Fairfield CT 06812-8923
orphan@rarediseases.org
www.rarediseases.org
Tel: 203-746-6518 800-999-NORD (-6673)
Fax:
203-746-6481
This
information is available courtesy of the National Institute of Neurological
Disorders and Stroke. It is in the public domain and can be downloaded, reproduced,
or copied without permission. For more information go to
http://www.ninds.nih.gov/health_and_medical/disorder_index.htm
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