CD-ROM Today 1996 January/CD-ROM Today 1996 January.iso/dp/0410/04108.txt

Text File | 1994-01-17 | 7.6 KB | 179 lines

$Unique_ID{BRK04108}
$Pretitle{}
$Title{Pick's Disease}
$Subject{Pick's Disease Diffuse Degenerative Cerebral Disease Lobar Atrophy
Alzheimer's Disease Huntington's Disease}
$Volume{}
$Log{}

Copyright (C) 1989, 1990 National Organization for Rare Disorders, Inc.

673:
Pick's Disease

** IMPORTANT **
It is possible that the main title of the article (Pick's Disease) is not
the name you expected.  Please check the SYNONYM listing to find the
alternate names and disorder subdivisions covered by this article.

Synonyms

     Diffuse Degenerative Cerebral Disease
     Lobar Atrophy

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

     Alzheimer's Disease
     Huntington'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.

Pick's Disease is a very rare progressive disease affecting the lobes of
the brain.  Major symptoms may include changes in intellect, behavior and
personality.

Symptoms

Pick's Disease is characterized by progressive deterioration of intellect
with changes in behavior and personality.   The memory is usually intact in
the early stages of the disease and there is less disorientation than in
Alzheimer's disease.  This brain disease is very closely related to
Alzheimer's disease and can even coexist with Alzheimer's.  People with
Pick's Disease don't show the tangles and plaques in the brain that are
characteristic of Alzheimer's.  However, in later stages there is loss of
motor control as well as confusion and severe dementia (loss of intellect).

Pick's Disease is characterized by atrophy (shrinkage) of the brain, but
the atrophy does not occur evenly over the entire brain.  Only the lobes of
the brain are affected while other areas of the brain appear unaffected.
There are also changes in certain nerve cells in the brain and the presence
of Pick's inclusion bodies.  The reason for this is not known.  The disease
primarily affects individuals in their 50's, 60's and 70's, but early cases
have been reported in people as young as 30 years of age.  Diagnosis of
Pick's disease is very difficult, and a neurologist should be consulted.

Causes

Most cases of Pick's Disease have been reported to occur for no apparent
reason.  However, some cases appear in families and this may indicate some
genetic predisposition for the disease.  So far researchers have not been
able to located a mode of genetic transmission for this disorder.  However,
research on Alzheimer's disease and related dementias may shed some light on
the cause of Pick's Disease.

Affected Population

Pick's Disease affects females more often than males.  The incidence in the
United States.  indicates a much lower prevalence than Alzheimer's Disease.
Studies from the University of Michigan have shown a dozen cases of Pick's
Disease in 35 years.  However, In Minnesota, Pick's Disease accounts for
about 4% of dementias.  This may well indicate geographical variance and
there may possibly also be an ethnic relationship.

Related Disorders

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

Alzheimer's Disease is a progressive disorder of the brain affecting
memory, thought and language.  Groups of nerve endings in the cortex of
people with Alzheimer's degenerate which disrupts the passage of
electrochemical signals between the cells.  These areas of degeneration are
called "plaques".  Changes known as "neurofibrillary tangles" also occur in
nerve cells of the brain's cortex.  The number of plaques and tangles appear
to be directly proportional to the disturbance in intellectual function and
memory.  Recently scientists discovered that Alzheimer's Disease can be
inherited through a dominant gene in at least ten percent of affected
individuals.  Some researchers believe symptoms may be caused by excessive
amounts of a protein that builds up in the brains of persons affected by this
disorder.  There is also abnormally low levels of the brain chemical
(neurotransmitter) acetylcholine in patients with this disorder.  (For more
information on this disorder, choose "Alzheimer" as your search term in the
Rare Disease Database).

Huntington's Disease is an inherited, progressively degenerative
neurological illness.  Those affected experience involuntary movements, loss
of motor control, changes in gait, loss of memory, and eventual loss of both
mental capability and physical control.  In general, the symptoms of HD first
appear between thirty and fifty years of age.  It runs a progressive course,
usually over a ten to twenty year period.  (For more information on this
disorder, choose "Huntington" as your search term in the Rare Disease
Database).

Dementia can be a symptom of many disorders mimicking Alzheimer's and
Pick's disease.  To learn more about these illnesses choose "Dementia" as
your search term in the Rare Disease Database).

Therapies:  Standard

The diagnosis of Pick's Disease is usually made by a neurologist after taking
a careful physical history and examination, a neurological examination,
psychometric testing, CAT scan and EEG testing.  Patients with progressive
dementia often experience frustration, anxiety, and depression resulting from
their inability to function at their previous level.  These frustrations can
be minimized by maintaining a stable home environment and a structured
routine that does not place excessive demands on the patient.  Sedatives or
other drugs that might further dull the patient's intellect should generally
be avoided.  Other treatment is symptomatic and supportive.

Therapies:  Investigational

The National Institute of Neurological Disorders and Stroke is seeking
patients with Pick's Disease and other types of frontal lobe atrophy to
participate in a neurobehavioral study.  For more information, physicians
should contact:

     Jordan Grafman, Ph.D., Chief
     Cognitive Neuroscience Section, Medical Neurology Branch
     NIH/National Institute of Neurological Disorders and Stroke (NINDS)
     Bldg. 10, Rm. 5C422
     Bethesda, MD  20892
     [phone]

This disease entry is based upon medical information available through
November 1990.  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 Pick's Disease, please contact:

     National Organization for Rare Disorders (NORD)
     P.O. Box 8923
     New Fairfield, CT  06812-1783
     [phone]

     Alzheimer's Disease and Related Disorders Association, Inc.
     National Headquarters
     919 N. Michigan Ave., Suite 1000
     Chicago, IL  60611
     [phone]
     [phone]

     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]

References

INTERNAL MEDICINE, 2nd Ed.:  Jay H. Stein, ed.-in-chief; Little, Brown and
Co., 1987.  Pp. 2150-2153.