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$Unique_ID{BRK03632}
$Pretitle{}
$Title{Creutzfeldt-Jakob Disease}
$Subject{Creutzfeldt-Jakob Disease Spastic Pseudosclerosis Jakob-Creutzfeldt
disease Corticostriatal-spinal degeneration CJD Subacute Spongiform
Encephalopathy Alzheimer's Disease}
$Volume{}
$Log{}
Copyright (C) 1984, 1985, 1987, 1988, 1990, 1991, 1992 National
Organization for Rare Disorders, Inc.
33:
Creutzfeldt-Jakob Disease
** IMPORTANT **
It and is possible that the main title of the article (Creutzfeldt-Jakob
Disease) is not the name you expected. Please check the SYNONYMS listing to
find the alternate name and disorder subdivisions covered by this article.
Synonyms
Spastic Pseudosclerosis
Jakob-Creutzfeldt disease
Corticostriatal-spinal degeneration
CJD
Subacute Spongiform Encephalopathy
Information on the following diseases can be found in the Related
Disorders section of this report:
Alzheimer's Disease
General Discussion
** REMINDER **
The Information contained in the Rare Disease Database is provided for
educational purposes only. It should not be used for diagnostic or treatment
purposes. If you wish to obtain more information about this disorder, please
contact your personal physician and/or the agencies listed in the "Resources"
section of this report.
Creutzfeldt-Jakob Disease is a rare disorder that is characterized by the
progressive degeneration of the central nervous system (spongiform
encephalopathy) and by neuromuscular disturbances. This disease is thought
to be transmissible in the form of an infectious particle known as a prion.
Creutzfeldt-Jakob Disease generally occurs in middle life.
Symptoms
The early stages of Creutzfeldt-Jakob Disease are characterized by memory
failures and behavioral changes. The patient may have difficulty in
concentrating, a lack of coordination and visual disturbances. Sudden
involuntary spasms of muscles (myoclonus) may occur. The toes may contract
in response to the irritation of the bottom of the foot (extensor plantar
reflexes) and there may be increased reflex reactions (hyperreflexia).
Creutzfeldt-Jakob Disease progresses to pronounced mental deterioration,
weakness on one side of the body (hemiparesis) and sensory disturbances.
There is a progressive wasting away of muscles (muscular atrophy). Patients
generally cease speaking (mutism) and movement becomes very slow or absent
(akinesia). Seizures and semi-coma may ensue. Death generally occurs within
a year, and may take place after only a few months.
This disease can produce characteristic changes in the electroence (EEG)
which is a test for brain wave activity. Computerized tomography (CT scan)
can demonstrate a deterioration of brain tissue.
Causes
Creutzfeldt-Jakob Disease is believed to be caused by a small infectious
particle called a prion (protein infectious agent). The mode of transmission
is not completely understood. About 10 percent of reported cases run in
families.
It has been suggested that prolonged therapy during the 1970's with
pituitary-derived human growth hormone (HGH) may have been a risk factor for
the development of Creutzfeldt-Jakob Disease. Further studies are required
to demonstrate this relationship and to attempt to define the duration of HGH
therapy that increased the risk of this disease. Today human growth hormone
is manufactured through biotechnology engineering (r-hGH) so transmission of
the Creutzfeldt-Jakob prion is no longer a risk with these recombinant
products.
Affected Population
Creutzfeldt-Jakob Disease affects both males and females. The disease occurs
most frequently around the age of 55 to 59. Incidence of the disease is very
low in the general population, but the exact prevalence is unknown.
Related Disorders
Symptoms of the following disorder can be similar to those of Creutzfeldt-
Jakob Disease. Comparison may be useful for a differential diagnosis:
Alzheimer's Disease is a common progressive condition of the brain
affecting memory, thought and language. The degenerative changes lead to the
formation of plaques or patches within the brain and the loss of cholinergic
neurotransmitter function. The early behavioral changes may be barely
noticeable, but as the disease progresses memory losses increase and there
are personality, mood and behavioral changes. There may also be disturbances
of judgment, concentration and speech along with confusion and restlessness.
(For more information on this disorder, choose "Alzheimer's Disease" as your
search term in the Rare Disease Database).
Therapies: Standard
The treatment of Creutzfeldt-Jakob Disease is symptomatic and supportive.
Patients should be guarded against infection and given as much support as
possible.
Therapies: Investigational
Possible drug treatments for Creutzfeldt-Jakob Disease are now being
evaluated. The testing of these drugs is being conducted on rodents and
primates (other than human) that have been induced with this disease.
Current clinical studies include tests of anti-viral drugs such as
amantadine, a compound useful in the treatment of Parkinsonism. Some
patients taking these drugs have experienced brief periods of improvement,
but prolonged treatment has not shown lasting benefit.
Scientists are conducting biochemical analyses of brain tissue, blood,
and serum from patients with Creutzfeldt-Jakob disease. To help with this
research, scientists are seeking biopsy and autopsy tissue, blood and
cerebrospinal fluid from patients with Creutzfeldt-Jakob and related
diseases. The following investigators have expressed an interest in
receiving such material:
Dr. Stephen DeArmond/Dr. Stanley Prusiner
Department of Pathology/Neuropathology Unit
HSW 430
University of California
San Francisco, CA 94143
(415) 476-5236
Dr. Clarence J. Gibbs/Dr. D. Carleton Gajdusek
NIH/National Institute of Neurological Disorders & Stroke
Laboratory of Central Nervous System Studies, Building 36, Room 4A-15
9000 Rockville Pike
Bethesda, MD 20892
(301) 496-4821
Dr. Elias Manuelidis
Yale University School of Medicine, Section of Neuropathology
333 Cedar Street
New Haven, CT 06510
(203) 785-4442
A study of early onset dementia occurring as a result of Creutzfeldt-
Jakob Disease is being conducted by the National Institute of Mental Health
(NIMH) and the Neuropsychiatric Research Hospital. This study includes a
thorough neuropsychological evaluation, advanced brain imaging and evaluation
using newly developed biochemical assay techniques. Participants in this
study must be under forty-five years of age and not require special medical
care. Physicians with patients who are interested should contact:
Denise Juliano, MSW
Coordinator of Admissions
Neuropsychiatric Research Hospital
2700 Martin Luther King Jr. Ave., SE
Washington, DC 20032
(202) 373-6100
An epidemiologic (affected population) study of Creutzfeldt-Jakob Disease
is being conducted at Loma Linda University under the supervision of Carey G.
Smoak. The project is funded by the National Institutes of Health.
Interested persons may call or write:
Creutzfeldt-Jakob Disease Study
Department of Neurology, Rm. 1580
School of Medicine
Loma Linda University
Loma Linda, CA 92350
(714) 799-2146
This disease entry is based upon medical information available through
September 1992. Since NORD's resources are limited, it is not possible to
keep every entry in the Rare Disease Database completely current and
accurate. Please check with the agencies listed in the Resources section for
the most current information about this disorder.
Resources
For more information on Creutzfeldt-Jakob Disease, please contact:
National Organization for Rare Disorders (NORD)
P.O. Box 8923
New Fairfield, CT 06812-1783
(203) 746-6518
Alzheimer's Disease and Related Disorders Association
National Headquarters
70 E. Lake Street
Chicago, Illinois 60601
(312) 853-3060
(708) 330-0230
(800) 621-0379 (In Illinois)
(800) 572-6037 (out of state)
NIH/National Institute of Neurological Disorders & Stroke (NINDS)
9000 Rockville Pike
Bethesda, MD 20892
(301) 496-5751
(800) 352-9424
For more information about long-term care facilities:
National Hospice Organization
1901 N. Fort Myer Drive, Suite 902
Arlington, VA 22209
(703) 243-5900
References
MENDELIAN INHERITANCE IN MAN, 10th Ed.: Victor A. McKusick, Editor: Johns
Hopkins University Press, 1992. Pp. 281-282, 927-929.
CECIL TEXTBOOK OF MEDICINE, 19th Ed.: James B. Wyngaarden, and Lloyd H.
Smith, Jr., Editors; W.B. Saunders Co., 1990. Pp. 2191-2193.
PRINCIPLES OF NEUROLOGY, 4th Ed.; Raymond D. Adams, M.D. and Maurice
Victor, M.D., Editors; McGraw-Hill Information Services Company, 1989. Pp.
609-611.
HUMAN GROWTH HORMONE AND CREUTZFELDT-JAKOB DISEASE, S. Zekauskas; J Okla
State Med Assoc (Sept 1990; 83(9)): Pp. 447-448.
CREUTZFELDT-JAKOB DISEASE IN PITUITARY GROWTH HORMONE RECIPIENTS IN THE
UNITED STATES, R. Thomson; JAMA (Feb. 1991; 20(265)): Pp. 880-884.
MOLECULAR BIOLOGY OF PRION DISEASES, S.B. Prusiner; Science (June 1991;
252(5012)): Pp. 1515-1522.