Actor Dudley Moore Receiving Treatment for Progressive Supranuclear Palsy (PSP), a Degenerative Parkinson's-Like Disease, at Kessler Institute for Rehabilitation

PSP Hits One in 100,000

Sep 29, 1999, 01:00 ET from Kessler Institute for Rehabilitation

    WEST ORANGE, N.J., Sept. 29 /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
     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.
     -- It is a rare degenerative brain disorder related to Parkinson's
     -- 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.
     -- 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.
     -- 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.
     -- 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.
     -- 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,
     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 the
     diagnosis 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