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$Unique_ID{BRK04087}
$Pretitle{}
$Title{Parkinson's Disease}
$Subject{Parkinson's Disease Parkinsonism (Paralysis Agitans; Shaking Palsy;
Secondary Parkinsonism; Symptomatic Parkinsonism; Postencephalitic
Parkinsonism; Drug-Induced Parkinsonism) Parkinsonism Dementia Complex
Juvenile Parkinsonism of Hunt (Hunt Corpus Striatum Syndrome; Pallidopyramidal
Syndrome) Hallervorden-Spatz Disease Benign Familial Tremor Benign Essential
Tremor Progressive Supranuclear Palsy Olivopontocerebellar Atrophy}
$Volume{}
$Log{}

Copyright (C) 1984, 1985, 1986, 1987, 1988, 1990, 1991, 1992, 1993
National Organization for Rare Disorders, Inc.

4:
Parkinson's Disease

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

Synonyms

     Parkinsonism (Paralysis Agitans; Shaking Palsy; Secondary Parkinsonism;
Symptomatic Parkinsonism; Postencephalitic Parkinsonism; Drug-Induced
Parkinsonism)

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

     Parkinsonism Dementia Complex
     Juvenile Parkinsonism of Hunt (Hunt Corpus Striatum Syndrome;
Pallidopyramidal Syndrome)
     Hallervorden-Spatz Disease
     Benign Familial Tremor
     Benign Essential Tremor
     Progressive Supranuclear Palsy
     Olivopontocerebellar Atrophy

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.


Parkinson's disease is a slowly progressive neurologic condition
characterized by involuntary trembling (tremor), muscular stiffness or
inflexibility (rigidity), slowness of movement and difficulty carrying out
voluntary movements.  Degenerative changes occur in areas deep within the
brain (substantia nigra and other pigmented regions of the brain), causing a
decrease in dopamine levels in the brain.  Dopamine is a neurotransmitter,
which is a chemical that sends a signal in the brain.  Parkinsonian symptoms
can also develop secondary to hydrocephalus (a condition in which the head is
enlarged and areas of the brain accumulate excessive fluids, resulting in an
increase in pressure on the brain), head trauma, inflammation of the brain
(encephalitis), obstructions (infarcts) or tumors deep within the cerebral
hemispheres and the upper brain stem (basal ganglia), or exposure to certain
drugs and toxins.  Parkinson's disease is slowly progressive and may not
become incapacitating for many years.

Symptoms

Parkinson's disease generally begins with a subtle slight tremor, especially
in the hands.  At first, the tremor occurs at rest, then becomes more
pronounced with fatigue and emotional stress, lessening during voluntary
movements.  The tremor may be limited to the arms or extend to the neck, jaws
and legs.  Voluntary movements such as walking become increasingly difficult.

Walking becomes slow, stiff and shuffling.  Perception, thinking
processes and sensation generally remain normal, although some patients may
experience a reduction of intellectual abilities (dementia).  The depression
that sometimes develops in Parkinson's disease may be part of the disease or
a reaction to it.

As the disease advances, a stooped posture and an immobile, unblinking
facial expression with frequent drooling develops.  Oily skin (seborrhea) may
be present on the face and scalp.  A feeling of being "frozen" in a position,
unable to make a voluntary movement, is a repeated symptom of Parkinson's
disease.

Causes

The cause of Parkinson's disease is unknown in most cases.  A few cases have
resulted from carbon monoxide or manganese poisoning.  Drug-induced
parkinsonian symptoms can develop from drugs used to treat psychiatric
disorders (dopamine-receptor antagonistic drugs).  These symptoms usually
disappear when the drugs are withdrawn or the dosage is decreased, or with
time during treatment.  A few families with multiple cases of Parkinson's
disease have been identified but a genetic basis has not been established.

Affected Population

Although 10 to 20 percent of all cases of Parkinson's disease are diagnosed
in individuals under the age of 40 years, this disorder occurs primarily in
the middle-aged and elderly population.  Between 300,000 and 500,000 cases of
classic Parkinson's disease are found in the United States.  As the National
Institute of Neurological Disorders and Stroke reported in a study of major
neurologic disorders in biracial populations, the occurrence of Parkinson's
disease shows no gender (male to female) or racial differences.

Related Disorders

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

Juvenile Parkinsonism of Hunt is an extremely rare hereditary syndrome
with onset during the teens, 20's or early 30's.

Parkinsonism Dementia Complex is associated with motor neuron disease
such as amyotrophic lateral sclerosis (ALS).

A rare form of Parkinson's-like syndrome has been found in western
Pacific Islands and has been determined to be caused by eating a locally
grown toxic bean.

A drug-induced Parkinsonism was identified in young heroin addicts who
abused a "designer drug" originally in a fairly localized community in
California.  Primates (i.e., monkeys) given the same toxic substance are
considered a study model for this disorder because parkinsonism can be
induced by the heroin substitute.

Benign Essential Tremor Syndrome is a disorder of the nervous system that
has no known cause.  It is characterized by a fine or coarse tremor, primarily
in the hands and head.  This disorder is similar to Benign Familial Tremor,
which tends to run in families.  Benign essential tremor syndrome may be
slowly progressive, eventually affecting other parts of the body.  When the
affected area of the body is in movement or voluntarily moved or held in one
position, the tremor is rhythmic and occurs at the rate of about 4 to 12
times per second.  This is in contrast to Parkinson's tremors, which usually
diminish or disappear entirely with purposeful movement.  The tremors mainly
affect the upper extremities and are aggravated by stress, anxiety, fatigue
and cold.  (For more information on this disorder, choose "Benign Essential
Tremor Syndrome" as your search term in the Rare Disease Database).

Hallorvorden-Spatz Disease affects movement.  It is associated with
degeneration of the nervous system.  A variety of symptoms can develop,
including muscular rigidity, stiffness, uncontrolled movement, contractions
(spasticity) and dementia.  Onset typically occurs during childhood, although
occasionally the disease appears in adulthood.  Imperfect articulation of
speech, mental retardation and facial grimacing are reported less frequently.
The clinical syndrome may resemble parkinsonism in some cases.  (For more
information on this disorder choose "Hallervorden-Spatz Disease" as your
search term in the Rare Disease Database).

Olivopontocerebellar Atrophy is a group of inherited forms of Ataxia
(impaired ability to coordinate movement).  It is characterized by
progressive neurologic degeneration (gradual deterioration) affecting certain
areas of the brain.  The loss of these brain cells (neurons) results in
impaired muscle coordination (ataxia), tremor, involuntary movement and
speech disturbance (dysarthria).  A wide variety in severity and age of onset
may be found in all types of Olivopontocerebellar atrophy.  (For more
information on this disorder, choose "Olivopontocerebellar Atrophy" as your
search term in the Rare Disease Database).

Progressive Supranuclear Palsy (PSP) is a neurologic disorder associated
with spastic weakness of muscles affected by the cranial nerves; i.e.,
muscles of the face, throat and tongue.  Onset of the disorder usually occurs
in middle age.  The first noticeable symptom of this disorder is usually loss
of balance while walking.  Patients may have unexplained falls or a stiff
awkwardness in the walk.  New symptoms can develop during the course of PSP,
and previously mild problems may become more severe with time.  (For more
information on this disorder, choose "Progressive Supranuclear Palsy" as
your search term in the Rare Disease Database).

Therapies:  Standard

With proper treatment, many Parkinson's disease patients may enjoy a normal
life span.  A number of drugs provide degrees of symptomatic relief.  These
include levodopa, anticholinergics and amantadine, a dopamine releasing agent
that acts in coordination with levodopa.  Anticholinergic agents such as
trihexyphenidyl, benztropine mesylate, biperiden and diphenhydramine help
control tremors and rigidity.  Amantadine hydrochloride helps reduce tremors
and rigidity and improves spontaneous movements.  Bromocriptine (Parlodel)
and/or pergolide (Permax) may be useful in some cases, particularly in
conjunction with other drugs such as the combination of levodopa and
carbidopa (Sinemet).

The treatment of choice is a combination of levodopa (the precursor of an
amino acid) and carbidopa (an enzyme inhibitor).  This drug (Sinemet) tends
to become less effective over time.  Some doctors have suggested that
patients try to prevent the "on-off" phenomenon by taking Sinemet an hour
before meals since protein is found in almost all foods composed of amino
acids which compete with levodopa for access to the brain.  However, since
many Parkinson's patients are debilitated due to the combined effects of the
disease and aging, and may also have difficulty eating regularly due to
tightened throat muscles, good nutrition is often difficult to maintain.
Therefore, depleting protein in the diet of Parkinson's patients could be
harmful or dangerous.  Proper diet will permit absorption of the drug by the
brain's receptor cells so that the protein in the meal will have much less
effect on the drug's usefulness.  If patients experience nausea with this
method, they can take the drug with soda crackers or a similar non-protein
snack.

The orphan drug deprenyl (Eldepryl) was approved for marketing by the FDA
in 1989.  This drug is a monoamine oxidase inhibitor which can be used in
late stage Parkinson's disease in combination with levodopa and carbidopa.
More recent research suggests that Eldepryl given to people with early stage
Parkinson's disease may significantly delay progression of more advanced
symptoms.

Physical therapy for Parkinson's patients may include exercises for
speaking, swallowing and overall muscle tone.  Exercises will not stop the
disease's progression, but may reduce disability.  Exercise can also improve
the emotional well-being of a patient.

Therapies:  Investigational

Additional dopamine agonist drugs are under investigation for Parkinson's
disease.  In the very early stages of experimentation is the implantation of
cells from the patient's own adrenal gland or from fetal tissue into the
brain to increase the amount of dopamine available to the affected
structures.  Animal studies continue to examine whether this procedure may
become more effective in humans with time.

Three studies published in the November 26th, 1992 issue of the New
England Journal of Medicine indicate that fetal cell transplants into brains
of people with Parkinson's Disease have resulted in moderate improvement of
symptoms.  Dosages of Parkinson's drugs were able to be reduced in most
patients.  However, more research is needed to determine the long-term
effectiveness and reduction of brain damage from the surgery.

Worldwide interest in drugs to treat Parkinson's disease is evident in
the various new therapies being developed.  In Italy, Farmitlalia Carlo Erba
is investigating the drug cabergoline to treat Parkinson's symptoms.  In
Japan, Thomai researchers are studying the Dopamin D-2 agonist talipexole.
In Israel, Teva Pharmaceuticals is developing TVP101 as a possible treatment.
Merrell-Dow is testing MDL72974A.  Hoffmann-LaRoche is studying the RO
and N-(2 aminoethyl)-5-chloro-2-pyridine carboxamide.  Ropinerole is being
developed by SmithKline Beecham.  Many of the trials are in early Phase I and
II stages.

The orphan product Apomorphine HCL injection is being tested for the
treatment of the on-off fluctuations associated with late-stage Parkinson's
Disease.  The product is manufactured by:

     Britannia Pharmaceuticals Ltd.
     Forum House, Brighton Rd.
     Redhill, Surrey, UK

This disease entry is based upon medical information available through
May 1993.  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 Parkinson's disease, please contact:

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

     Parkinson's Disease Foundation
     Columbia Presbyterian Hospital
     Neurologic Institute
     710 W. 168th St.
     New York, NY 10032
     [phone]

     United Parkinson Foundation
     360 W. Superior St.
     Chicago, IL 60610
     [phone]

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

     International Tremor Foundation
     360 W. Superior St.
     Chicago, IL  60610
     [phone]

References

THE MERCK MANUAL 15th ed:  R. Berkow, et al:  eds; Merck, Sharp & Dohme
Research Laboratories, 1987.  P. 1421.

CECIL TEXTBOOK OF MEDICINE, 18th ed.:  James B. Wyngaarden, and Lloyd H.
Smith, Jr., Eds.:  W.B. Saunders Co., 1988.  Pp. 2143-7.

MENDELIAN INHERITANCE IN MAN, 9th Ed.:  Victor A. McKusick, Editor:
Johns Hopkins University Press, 1990.  Pp. 707, 710-1, 1409, 1703.

PRINCIPLES OF NEUROLOGY, 4th ed.:  Raymond D. Adams, and Maurice Victor:
McGraw Hill, Inc., 1989.  Pp. 940-1.

UNILATERAL TRANSPLANTATION OF HUMAN FETAL MESENCEPHALIC TISSUE INTO THE
CAUDATE NUCLEUS OF PATIENTS WITH PARKINSON'S DISEASE:  D.D. Spencerr, et al.;
The New England Journal of Medicine; (November 26, 1992, issue 327 (22)).
Pp. 1541-47.

SURVIVAL OF IMPLANTED FETAL DOPAMINE CELLS AND NEUROLOGIC IMPROVEMENT 12
TO 42 MONTHS AFTER TRANSPLANTATION FOR PARKINSON'S DISEASE  C.R. Freed, M.D.,
et al.; The New England Journal of Medicine; (November 26, 1992, issue 327
(22)).  Pp. 1549-55.

BILATERAL FETAL MESENCEPHALIC GRAFTING IN TWO PATIENTS WITH PARKINSONISM
INDUCED BY 1-METHYL-4PHENYL-1,2,3,6-TETRAHYDROPYRIDINE:  Hakan Widner, M.D.,
et al.; The New England Journal of Medicine; (November 26, 1992, issue 327
(22)).