peter dawkins wrote:
>One of Sydney's leading neurologists is very interested in the Dudley
Moore
>listing of last week. Could anybody list it again please.
Company Press Release
Actor Dudley Moore Receiving Treatment for Progressive Supranuclear
Palsy (PSP), a Degenerative Parkinson's-Like Disease, at Kessler
Institute for Rehabilitation
http://biz.yahoo.com/prnews/990929/nj_kessler_1.html
PSP Hits One in 100,000
WEST ORANGE, N.J., Sept. 29, 1999 /PRNewswire/ -- Actor, composer and
pianist Dudley Moore is being treated for Progressive Supranuclear Palsy
(PSP) at Kessler Institute for Rehabilitation. PSP is a rare,
degenerative brain disorder related to Parkinson's disease.
Due to the slow, progressive nature of this disease, Moore, 64, was only
recently diagnosed after suffering from symptoms for over five years.
Dudley Moore said, ``I have decided to come forward to tell my friends
and the public that I am being treated for a disease closely related to
Parkinson's. I hope that by revealing my illness today, I can bring
attention to this rare malady that afflicts thousands of people, many of
whom remain undiagnosed. I wish to raise public awareness of the
critical need for medical research and education on behalf of all those
with this disease who struggle to continue leading productive lives. I
would also like to thank my dedicated physicians who are working to help
slow the progression of my symptoms.''
A team of doctors who specialize in this rare disorder is currently
treating Mr. Moore. The team includes Dr. Lawrence Golbe of UMDNJ-Robert
Wood Johnson Medical School, New Brunswick, NJ; Dr. Stephen Reich of the
Johns Hopkins School of Medicine, Baltimore, MD; Dr. Martin Gizzi, of
the NJ Neuroscience Institute at JFK Medical Center, Edison, NJ; and Dr.
Thomas Galski, at Kessler Institute for Rehabilitation.
Also known as Steele-Richardson-Olszewski Syndrome, PSP is a rare
disorder striking late-middle-aged adults. Dr. Reich said, ``Because the
earliest signs of PSP are often quite subtle, and sometimes take several
years to evolve, the diagnosis is typically delayed. At the onset of
symptoms, patients are often misdiagnosed as having Parkinson's disease,
Alzheimer's disease, strokes, alcohol intoxication, or even inner ear
disease.'' Early symptoms include falling, difficulty walking,
imbalance, and slow movements similar to Parkinson's disease. People who
have PSP may also experience vision problems, as well as difficulty with
speech and swallowing. Its cause remains a mystery, and while there is
no cure, many of the symptoms can be alleviated by therapy, medication
and other interventions.
His doctors agree that Mr. Moore is in the early stages of this disease.
He is able to walk short distances without assistance, but prefers the
use of a cane for stability. He is in good spirits with normal mental
functioning and is maintaining a regular daily routine.
About his condition, and in a lighter moment, Mr. Moore continued, ``I
understand that one person in 100,000 suffers from this disease, and I
am also aware that there are 100,000 members of my union, the Screen
Actors Guild (SAG), who are working every day. I think, therefore, it is
in some way considerate of me that I have taken on the disease for
myself, thus protecting the remaining 99,999 SAG members from this
fate.''
Under the supervision of Dr. Thomas Galski, Director of Neuropsychology
Services at Kessler Institute, Mr. Moore is currently undergoing a
comprehensive therapy program aimed at improving cognitive and physical
functioning. He also is receiving experimental drug therapy.
FACTS ABOUT PROGRESSIVE SUPRANUCLEAR PALSY
-- It is a rare degenerative brain disorder related to Parkinson's
disease.
-- Strikes middle-aged adults, with 64 as the median age of onset.
-- Attacks 1.4 in 100,000 people, slightly more men than women.
-- First identified as a distinct neurological disorder in 1963 by Dr.
John C. Steele, Dr. J.C. Richardson and
Dr. J Olszewski.
WHAT ARE THE SYMPTOMS?
-- Symptoms typically include falling, difficulty walking, imbalance,
slow movements, vision problems,
difficulty speaking, problems swallowing food, changes in mood or
behavior. Stiffness in muscles follows initial
symptoms of falling in many cases.
WHAT CAUSES PSP?
-- The symptoms are caused by gradually progressive damage to a group of
cells in part of the brain
called the "midbrain." These cells are involved in eye-movements
and balance. The cause of the
degeneration of these cells is not known.
IS THERE A PROVEN EFFECTIVE TREATMENT?
-- No. There is no drug known that will stop the inexorable progression
of the disease. Certain drugs
used to treat Parkinson's disease are used to improve the symptoms
of PSP. Doctors and researchers
are studying a variety of drugs to search for effective treatments.
WHAT SHOULD PEOPLE DO IF THEY SUSPECT THEY MAY HAVE PSP?
-- People should seek treatment from a neurologist, as well as
contacting the Society for PSP at the Woodholme
Medical Building, 1838 Greene Tree Rd., Suite 515, Baltimore, MD
21208. Phone: 800-457-4777,
http://www.psp.org.
STATEMENTS FROM DOCTORS TREATING DUDLEY MOORE
Dr. Thomas Galski, of Kessler Institute for Rehabilitation, East Orange,
NJ
Dudley Moore has been undergoing a comprehensive program of
rehabilitation at Kessler Institute that has
emphasized treatments to help with problems in balance and gait,
expressive speech, vision and swallowing.
The program has also involved treatment to help Mr. Moore cope with the
current and anticipated impact of PSP
on his life. He has been a regular and active participant in all of his
treatment components. Indeed, he works
very diligently in this exhausting program of rehabilitation. Dudley is
completely dedicated to maximizing his
functioning.
Contact: Kathy Lewis, 973-243-8503
Dr. Stephen Reich, of the Johns Hopkins School of Medicine, Baltimore,
MD
I admire Mr. Dudley Moore for having the courage to go public with
thediagnosis of Progressive Supranuclear
Palsy (PSP). By doing so, he not only brings attention and additional
support to his own plight, but
also brings wider attention to the orphan disease, PSP. I saw Mr. Moore
for the first time in January, 1998, at
which point it was clear that something neurological was going on but
that something was not clear. By
the time I saw him in follow-up, in February 1999, the diagnosis of
Progressive Supranuclear Palsy was
apparent. In the meantime, the most important clue about the diagnosis
came from Drs. Martin Gizzi and
Michael Rosenberg at the New Jersey Neuroscience Institute. Using a
technique known as infrared video
oculography, they were able to measure the speed of Mr. Moore's eye
movements. This demonstrated that his
vertical eye movements were very slow and this is one of the earliest
features of PSP, but often difficult to
appreciate clinically.
Progressive Supranuclear Palsy is known as a parkinsonian syndrome and
is best thought of as a "cousin" of
Parkinson's disease. It typically begins with impaired balance, gait
and falls. Other features include
difficulty talking and swallowing, with the most characteristic sign
being limited up and down movements of the
eyes, known as vertical ophthalmoparesis. Because the earliest signs of
PSP are often quite subtle, and
sometimes take several years to evolve, the diagnosis is typically
delayed.
Although PSP is not curable, many symptoms of the disease can be
improved with medications, rehabilitation,
and other interventions. The course of the disease is slowly
progressive and variable. Fortunately, through the
efforts of The Society for Progressive Supranuclear Palsy, which is
based in Baltimore, along with neurologists
and neuroscientists over the past decade, PSP has been the subject of
growing research, making all of
us involved with this disease optimistic that in the future we will have
a better understanding of its cause and with
it, improved therapies.
Contact: Gary Stepheenson, 410-955-5384
Dr. Lawrence Golbe, UMDNJ-Robert Wood Johnson Medical School, New
Brunswick, NJ
Progressive Supranuclear Palsy has been diagnosed in about 5,000
Americans and is present in about another
15,000 who have not been correctly diagnosed. PSP starts in the early
60s, on average. Its main
symptoms are loss of balance with falls; difficulty moving the eyes,
particularly up and down; slurred speech;
difficulty swallowing; stiffness of the neck and back; and difficulty
with some aspects of mental function. It
typically produces a chair-bound state 5 or 6 years into the illness.
PSP differs from Parkinson's disease in that
there is little or no tremor and little stiffening of the limbs. PSP
differs from Alzheimer's disease in that there is
little or no difficulty with memory, word finding or spatial
orientation. Rather, the problems are in the areas of
speed, inhibition, emotion and organization of thought.
Although fewer than one in 100 patients with PSP knows of another case
in the family, there is now evidence of a
subtle genetic component to the cause of the disease, the PSP-related
gene codes for a protein called
"tau", which helps maintain the structure of brain cells. Tau protein
accumulates in clumps in brain cells. The
steps from the defect in the tau gene to the clumping of tau protein and
loss of brain cells are now being studied
intensively by researchers.
There are presently two treatment trials for PSP in progress. PSP may
respond to physical therapy and speech
or swallowing therapy.
Mr. Moore has been seeing me for care of his PSP since May 1999 at the
Movement Disorders Center at Robert
Wood Johnson Medical School in New Brunswick. He is presently enrolled
in our double-blind trial of
the drug pramipexole, which is suspected of being able to slow the rate
of progression of the brain cell
degeneration in PSP. I am performing the study in collaboration with
Yale University School of Medicine,
where an experimental type of brain scan called "beta-CIT SPECT"
monitors the function of the brain cells that
are affected in PSP.
Contact: Susan Preston, 973-972-7265
Dr. Martin Gizzi, of the JFK Medical Center, Edison, NJ
Providing a correct diagnosis for Progressive Supranuclear Palsy can be
difficult because it is a rare condition that progresses slowly with
subtle symptoms. We have been working on the diagnosis and treatment of
PSP since the 1980s, when I began developing methods to distinguish
Parkinson's disease from PSP using recordings of eye movements. PSP is
characterized by extremely slow eye movements and loss of the ability to
look downward. Individuals with PSP may also experience balance
difficulties.
Dudley Moore was referred to the New Jersey Neuroscience Institute in
February of 1998, after he began experiencing balance problems. Because
PSP represents less than 5% of patients with Parkinson's-like features
and it may progress slowly, it is not diagnosed correctly for years, in
many cases. Being a concert pianist, Mr. Moore became aware of the
symptoms quite early when he experienced slowing and incoordination of
fine finger movements. Because he had so few signs of the disease, an
accurate diagnosis eluded physicians until we became aware of his slowed
eye movements. He was then referred back to Dr. Stephen Reich at Johns
Hopkins for treatment and monitoring for progression of the disease.
CONTACT: Peter Haigney, 732-632-1530
SOURCE: Kessler Institute for Rehabilitation
Copyright © 1999 PRNewswire.
--
Judith Richards, London, Ontario, Canada
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