CD-ROM Today 1996 January/CD-ROM Today 1996 January.iso/dp/0344/03448.txt

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$Unique_ID{BRK03448}
$Pretitle{}
$Title{Alzheimer's Disease}
$Subject{Alzheimer's Disease Presenile Dementia Senility Pick's Disease
Binswanger Disease Creutzfeldt-Jakob Disease}
$Volume{}
$Log{}

Copyright (C) 1984, 1985, 1986, 1987, 1988, 1989, 1990, 1991, 1992
National Organization for Rare Disorders, Inc.

29
Alzheimer's Disease

** IMPORTANT **
It is possible that the main title of the article Alzheimer's 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

     Presenile Dementia
     Senility

Information on the following diseases can be found in the Related
Disorders section of this report:

     Pick's Disease
     Binswanger Disease
     Creutzfeldt-Jakob 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.


Alzheimer's Disease is a progressive condition of the brain affecting
memory, thought and language.  The degenerative changes of Alzheimer's
Disease lead to patches or plaques in the brain and the entanglement of nerve
fibers (neurofibrillary tangles).  Memory loss and behavioral changes occur
as a result of these changes in brain tissue.

Symptoms

Alzheimer's Disease is a slow progressive illness.  The early behavioral
changes may not be noticed, especially difficulty with short term memory.  As
the disease progresses, memory loss increases and there are changes in
personality, mood and behavior.  Disturbances of judgment and concentration
occur, along with confusion and restlessness.  The type, severity, sequence,
and progression of mental changes vary widely.  Long periods with little
change are common, although occasionally the disease can be rapidly
progressive.

People with Alzheimer's Disease should be given regular physical
examinations to detect other organic disorders that may develop.  These
patients may be unable to communicate clearly regarding the development of
new or unrelated symptoms.

Causes

Alzheimer's Disease is inherited as an autosomal dominant trait in at least
10 percent of the cases.  Researchers studying the genetic forms of
Alzheimer's Disease have located defects causing the disorder on at least
three chromosomes.  These include genes on chromosomes 14, 21, and 19.  It is
not understood whether these genetic defects can cause different types of
Alzheimer's Disease or variations of the same disorder.  In other cases of
Alzheimer's Disease the cause is unknown.

Human traits, including the classic genetic diseases, are the product of
the interaction of two genes, one received from the father and one from the
mother.  In dominant disorders a single copy of the disease gene (received
from either the mother or father) will be expressed "dominating" the other
normal gene and resulting in the appearance of the disease.  The risk of
transmitting the disorder from affected parent to offspring is fifty percent
for each pregnancy regardless of the sex of the resulting child.

Alzheimer's Disease frequently occurs in individuals with Down Syndrome
who live past 35 years of age.  Chromosome 21 abnormalities are common to
both Down Syndrome patients and some of the familial Alzheimer's Disease
patients.

Some studies suggest that the disease may not be a single illness and
that several factors are involved.  Researchers at the UCLA Medical School
found that 100 percent of men with early Alzheimer's Disease (before the age
of 60) had the protein HLA-A2 on the surface of their white blood cells,
compared to 30 percent of healthy men under the age of 60, and 40 percent of
men with late-onset disease.  It is suggested that HLA-A2 positive men may be
at higher risk for early-onset Alzheimer's Disease.

Researchers at the John Hopkins Hospital are studying the brain tissue of
deceased Alzheimer's Disease patients, and have found nerve cell (neuronal)
degeneration.  These nerve cells are believed to contain the neurotransmitter
acetylcholine.  Some patients with Alzheimer's Disease have a 90 percent loss
of these cells.  There may be abnormally low levels of acetylcholine in the
brains of Alzheimer's Disease patients.

Researchers believe that brain cells normally produce a soluble form of
the amyloid protein.  However, people with Alzheimer's Disease have insoluble
deposits of amyloid (plaques) in their brain.  Therefore, an unknown factor
may be responsible for the plaque formation.  It may not be the amyloid
protein that causes Alzheimer's Disease, but instead another factor that
causes abnormal deposits of amyloid to occur.

Affected Population

Alzheimer's Disease occurs in approximately 2 percent to 3 percent of the
general population over 60 years of age.  Approximately 2.5 million people in
the United States are affected.  The disease affects more females than males
and a higher percentage of Afro-Americans than Caucasians.

Related Disorders

Symptoms of the following disorders can be similar to those of Alzheimer's
Disease.  Comparisons may be useful for a differential diagnosis:

Pick's Disease is a degenerative neurological disorder that affects the
frontal and temporal lobes of the brain.  The symptoms of Pick's Disease
closely resemble those of Alzheimer's Disease.  In the early stages, memory
is still intact and there is a high degree of disorientation.  In the later
stages of this disorder, there is a loss of motor control and language
skills.  There may also be severe dementia.  (For more information on this
disorder, choose "Pick's Disease" as your search term in the Rare Disease
Database).

Binswanger Disease is a form of senile dementia that is associated with
degenerative changes in the white matter of the brain.  The major symptoms
include the loss of short term memory, difficulty coping with unusual events,
self-centeredness and childish behavior.  Other symptoms may include urinary
incontinence, difficulty walking, tremor and depression.  (For more
information on this disorder, choose "Binswanger " as your search term in the
Rare Disease Database).

Creutzfeldt-Jakob Disease is a rare, degenerative disease of the brain
that is characterized by the progressive degeneration of the central nervous
system and by neuromuscular disturbances.  The early symptoms include a
marked loss of memory, behavioral changes, difficulty in concentration and
visual disturbances.  Sporadic, shock-like contractions of muscles
(myoclonus) may also be present.  The illness progresses to mental
deterioration, sensory disturbances and the progressive wasting away of
muscle.  (For more information on this disorder, choose "Creutzfeldt-Jakob"
as your search term in the Rare Disease Database).

Therapies:  Standard

The treatment of Alzheimer's Disease is symptomatic and supportive.
Tranquilizers may decrease agitation, anxiety or unusual behaviors.  The
depression often accompanying this illness may be treated with various
antidepressant drugs.  Proper diet and fluid intake are important.  Special
diets or food supplements may or may not be of benefit.  Exercise and
physical therapy may be helpful for some patients.  Patients with Alzheimer's
Disease should avoid drinking alcoholic beverages since alcohol can add to
the confused mental state.  The daily routine of a patient should be
maintained as normally as possible.  Continuation of social activities should
be encouraged.

The only drug approved for the treatment of Alzheimer's Disease is
Hydergine-LC.  Recent tests suggest that this medication may be ineffective
in treating Alzheimer's Disease patients.

Genetic counseling may be of benefit for patients and their families.

Therapies:  Investigational

Diagnosed Alzheimer's Disease patients who have one or more relatives (living
or deceased) also diagnosed with the disease, may participate in a clinical
research study being conducted at the National Institute of Neurological
Disorders and Stroke (NINDS) in Bethesda, MD.  The goal of the study is to
identify the gene linked to the type of Alzheimer's Disease that runs in
families.  Physicians who wish to refer potential candidates from families
with many affected individuals should contact:

     Ms. Linda Nee, M.S.W. or
     Dr. Ronald Polinsky
     NIH/National Institute of Neurological Disorders and Stroke (NINDS)
     Medical Neurology Branch, Bldg. 10, Rm. 5N236
     Bethesda, MD  20892
     [phone]

Once the defective gene for Alzheimer's Disease on chromosome 21 is
identified, the biochemical abnormality may be defined and researchers may
then learn how to correct the defect through drugs, surgery, environmental or
genetic manipulation.

Studies on the drug tetrahydroaminoacridine (THA or Tacrine) for
treatment of Alzheimer's Disease are presently underway.  It is hoped that
Tacrine may help small amounts of acetylcholine to stay in the brain for
longer periods of time.  This would not stop the progression of Alzheimer's
Disease, but may help temporarily improve symptoms such as memory loss and
confusion.  Studies published in 1992 indicate that Tacrine may produce
slight short-term benefits in a subcategory of Alzheimer's patients, but it
can also cause frequent side effects, primarily liver dysfunction and
gastrointestinal symptoms.  For more information on this drug, please
contact:

     Warner-Lambert Co.
     2800 Plymouth Road
     P.O. Box 1047
     Ann Arbor, MI 48106.

A study of early onset dementia occurring as a result of Alzheimer's
Disease is being conducted by the National Institute of Mental Health (NIMH)
and the Neuropsychiatric Research Hospital at Washington, D.C.  This study
includes a thorough neuropsychological evaluation, 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
     [phone]

The narcotic antagonist drug Naltrexone is being tested for treatment of
Alzheimer's Disease.  This drug may alleviate some symptoms.  Further testing
is required to determine long-term safety and effectiveness.

The drug Desferal administered intramuscularly twice daily is being
investigated as a treatment for Alzheimer's Disease.  The drug is
manufactured by Ciba-Geigy.  More study is needed to determine the long-term
safety and effectiveness of this treatment.

New drugs being developed for the treatment of Alzheimer's Disease by
Hoeschst Co. include:  Velnacrine (Metane), Suronacrine (HP 128), HP 749,
Ebiratide (HOE 427), HOE 065, and CAS 493.

The drug linopirine is being tested on Alzheimer's Disease patients.  It
acts "encourage" brain cells to increase production of acetylcholine that is
in short supply in the brains of persons with Alzheimer Disease.  Du Pont
Merck Pharmaceutical Co. is the manufacturer of this drug.

Other drugs in development for the possible treatment of Alzheimer's
Disease include:  Capoten, SQ 29852, HP290, Nimotop, Guanfacine, Zacopride,
Milacemide, Alcar, Oxiracetam, Avan, and Cognex.

Research on genetic diseases and their causes is ongoing.  The National
Institutes of Health (NIH) is sponsoring the Human Genome Project which is
aimed at mapping every gene in the human body and learning why they sometimes
malfunction.  It is hoped that this new knowledge will lead to prevention and
treatment of genetic disorders in the future.

This disease entry is based upon medical information available through
December 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 Alzheimer's 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, Inc.
     National Headquarters
     70 East Lake Street
     Chicago, IL  60601
     [phone]
     [phone]
     [phone] (In Illinois)
     [phone] (out of state)

     NIH/National Institute of Neurological Disorders & Stroke (NINDS)
     9000 Rockville Pike
     Bethesda, MD  20892
     [phone]
     [phone]

     NIH/National Institute on Aging (NIA)
     9000 Rockville Pike
     Bethesda, MD  20892
     [phone]

For Genetic Information and Genetic Counseling Referrals:

     March of Dimes Birth Defects Foundation
     1275 Mamaroneck Avenue
     White Plains, NY 10605
     [phone]

     Alliance of Genetic Support Groups
     35 Wisconsin Circle, Suite 440
     Chevy Chase, MD  20815
     (800) 336-GENE
     [phone]

References

THE MERCK MANUAL 15th ed. R. Berkow, et al:  eds; Merck, Sharp & Dohme
Research Laboratories, 1987.  P. 1337.

MENDELIAN INHERITANCE IN MAN, 10th Ed.:  Victor A. McKusick, Editor:
Johns Hopkins University Press, 1992.  Pp. 57-61.

CECIL TEXTBOOK OF MEDICINE, 19th Ed.:  James B. Wyngaarden, and Lloyd H.
Smith, Jr., Editors; W.B. Saunders Co., 1990.  Pp. 2075-2079.

PRINCIPLES OF NEUROLOGY, 4th Ed.; Raymond D. Adams, M.D. and Maurice
Victor, M.D., Editors; McGraw-Hill Information Services Company, 1989.  Pp.
923-926.

LACK OF EFFICACY OF HYDERGINE IN PATIENTS WITH ALZHEIMER'S DISEASE,
Thompson, Troy, et al.; N. Eng J Med (August 16, 2990, issue 323 (7)).  Pp.
445-448.

THERAPEUTIC FRONTIERS IN ALZHEIMER'S DISEASE, S.W. Miller et al.;
Pharmacotherapy (1992; 12(3)).  Pp. 217-231.

DRUG TREATMENT OF ALZHEIMER'S DISEASE, J.K. Cooper; Arch Intern Med (Feb
1991; 151(2)). Pp. 245-249.

ALZHEIMER'S DISEASE.  RECOGNIZING AND TREATING A FRUSTRATING CONDITION,
W.P. Shelton et al.; Postgrad Med (Sept 1991; 90(4)).  Pp. 33-34, 37-41.

 A DOUBLE-BLIND, PLACEBO-CONTROLLED MULTICENTER STUDY OF TACRINE FOR
ALZHEIMER'S DISEASE:  K.L. Davis, et al.; N Engl J Med, (October 29, 1992;
issue 327 (22).  Pp. 1253-9.