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Ken

>

>

> May 14, 2002

>

> Parkinson's `Clusters' Getting a Closer Look

>

> By MARY DUENWALD

>

> ichael J. Fox's celebrity can do more than raise money for

Parkinson's

> research. It may also help open an avenue of research that

scientists have

> long wanted to explore.

>

> Mr. Fox, it turns out, was one of four people who worked on a

production crew

> at a television studio in Vancouver, British Columbia, in the late

1970's and

> developed Parkinson's disease. Given that only 125 people worked on

the crew

> in those years †" including actors, directors, writers, production

people and

> technicians †" the number of cases seems extraordinary.

>

> It is possible that the cluster of cases arose purely from chance.

But

> researchers are interested in studying them in hopes they will lead

to clues

> to possible environmental factors or even viruses that may

contribute to to

> the disease.

>

> Typically, Parkinson's disease afflicts one in 300 people. In

people as young

> as J. Fox, 30 when the disease was diagnosed in 1991, the

illness is

> much rarer. Fewer than 5 percent of Parkinson's patients develop

symptoms

> before age 50, said Dr. Caroline Tanner of the Parkinson's

Institute. The

> Vancouver cluster includes Mr. Fox and a woman who learned she had

> Parkinson's at age 38.

>

> The four people worked together from 1976 to 1980, when it is

possible that

> the disease began in all of them. Parkinson's progresses gradually,

taking 5

> to 10 years from the time it starts to the appearance of the first

symptoms †"

> usually, rigidity in an arm or leg or tremor in a hand.

>

> Dr. Calne, director of the neurodegenerative disorders

center at the

> University of British Columbia, estimates that the odds of the four

cases

> occurring at the same time in such a small group of people are less

than 1 in

> 1,000.

>

> He and other scientists say the cluster warrants investigation.

>

> " I would definitely want to look further, " said Dr. J.

Langston,

> director of the Parkinson's Institute in Sunnyvale, Calif., and

chief

> scientific adviser to the J. Fox Foundation for Parkinson's

Research.

>

> Dr. Katrina Gwinn-Hardy, a Parkinson's specialist at the National

Institute

> of Neurological Disorders and Stroke, said, " It's intriguing and

worth

> pursuing. "

>

> Mr. Fox said he was curious about the cluster, out of both empathy

for the

> people he worked with and because, as he put it, " the beginning of

my career

> and the end of my career being tied in a single event is pretty

cosmic or

> weird to me. "

>

> But whether the cluster should be investigated, he said, is a

question he

> leaves to the judgment of scientists.

>

> Clusters of Parkinson's cases occur from time to time, when, for

example, a

> number of people in a neighborhood or small town develop the

disease. But

> they often go unnoticed or are ignored because scientists lack the

time and

> money to look into them. In this case, the publicity surrounding

Mr. Fox's

> admission that he had Parkinson's, nearly three and half years ago,

drew the

> cases into the spotlight.

>

> Don S. , who directed Mr. Fox in two Canadian situation

comedies

> beginning when the actor was 16, and who also has Parkinson's,

tried to get

> in touch with Mr. Fox soon after reading the news. " But I couldn't

get past

> his handlers, " he said.

>

> Mr. (also a cast member of " The X Files " for four years)

learned of

> his illness nine years ago, when he was 55.

>

> Sally Gardner, whose Parkinson's was diagnosed when she was 38, in

1984, had

> been a script supervisor in the late 1970's, and had worked with

Mr. Fox and

> Mr. . The fourth member of the cluster, a cameraman who has

kept his

> identity secret, was recently found by Jerry , writer and

director of

> a Canadian television documentary about Mr. Fox and Parkinson's

disease. The

> cameraman's diagnosis came at age 54.

>

> 's documentary, " The Parkinson's Enigma, " which was

broadcast last

> month, publicized the cluster's existence.

>

> Could something at the television studio have caused the disease in

all four

> people? Dr. Calne, Dr. Langston and other experts believe it could

have.

> Perhaps something they breathed or ate or drank †" a toxin,

perhaps, or an

> infectious agent †" set the disease process in motion.

>

> " If this is a genuine cluster and not a statistical fluke, " said

Dr. Oliver

> Sacks, a neurologist and writer, " it would certainly suggest an

environmental

> agent at work. "

>

> The mystery is especially compelling because scientists do not know

what

> causes most cases of Parkinson's. Most believe that both genetic

and

> environmental factors are at work. " We often say that maybe people

have some

> gene that predisposes them to be susceptible to any number of

things in the

> environment, " said Dr. J. Weiner, chairman of neurology

department at

> the University of land School of Medicine. " But's that's

probably just

> another way of saying we don't know the cause. "

>

> The disease occurs when cells in the substantia nigra, a darkly

pigmented

> part of the midbrain, about half the size of an adult index

fingernail, start

> to die off. These cells produce dopamine, a chemical messenger that

is

> essential for normal muscle movement. The cell death occurs

gradually, and

> that is why Parkinson's can go unnoticed so long.

>

> Once dopamine production declines by about 80 percent, the patient

begins to

> experience the four classic symptoms: tremor, stiffness, slow

movement and

> problems with walking, posture and balance. In some cases, the cell

death is

> set off by genetic mutations. Scientists have identified two genes

that are

> involved in Parkinson's disease and have pinpointed the locations

of four

> others.

>

> But Parkinson's does not seem to be primarily a genetic disorder.

It runs in

> the families of only about 10 percent to 15 percent of patients,

Dr. Tanner

> of the Parkinson's Institute said. A large study she conducted

indicated that

> the identical twins of Parkinson's patients are no more likely to

have the

> disease than fraternal twins †" a sign that the disease is not

largely

> genetic.

>

> Environmental agents have also been known to create Parkinson's

symptoms. In

> the era of World War I, for example, some people who had contracted

the virus

> that causes sleeping sickness later developed what came to be known

as

> post-encephalitic parkinsonism, a particularly severe disorder that

left

> people in trancelike states. Dr. Sacks's book " Awakenings " told how

these

> patients were able to regain movement when he treated them with

levodopa, a

> drug that is still the main treatment in Parkinson's. (Levodopa,

also known

> as L-dopa, is converted to dopamine in the brain.)

>

> It is possible that a virus could also have been involved in the so-

called

> Fox cluster. " It is important to look for infectious as well as

toxic

> agents, " Dr. Sacks said.

>

> Dr. Calne suspects a virus may indeed have caused the Parkinson's

cases in

> Vancouver. " In many areas of medicine it is fully accepted that

there are

> several types of cause for one type of disorder, " he said. " For

example,

> everyone recognized that cancer can be caused by genes, chemical

agents or

> infective agents. In the field of neurodegeneration, people are

often

> reluctant to draw the same general conclusion, in spite of

compelling

> evidence. "

>

> Perhaps, Dr. Calne said, Parkinson's can be caused by a single

event, when an

> infection or a toxic substance attacks cells in the substantia

nigra. Some

> cells are killed instantly; others are injured and die off over

time, causing

> the patient's symptoms to worsen progressively.

>

> The idea that viruses can cause Parkinson's, he said, is bolstered

by

> evidence that the disease has been somewhat more prevalent among

people who

> have worked in certain fields, including teachers, doctors,

dentists, nurses,

> loggers and miners. These people all had relatively high exposure

to

> infections †" teachers and doctors because of the people they

worked with and

> loggers and miners because they slept in close quarters in work

camps.

>

> Mr. does not recall any particular illness he suffered

while working

> on the television production crew. " You know what it's like with

flu and

> colds and things; they're not outstanding events, " he said.

>

> Toxins can also set off parkinsonism, as doctors learned in 1983,

when seven

> young adults in the San Francisco Bay Area suddenly developed

symptoms. Dr.

> Langston examined this cluster of patients and learned that they

all had

> injected themselves with a synthetic form of heroin that contained

the toxic

> molecule MPTP. The MPTP killed large numbers of the victims'

> dopamine-producing cells.

>

> Dr. Calne has already been in touch with the production studio

about starting

> an investigation in the cluster. " I'm not optimistic about finding

a cause

> after all this time, " he said, " but I think we might be able to

find some

> features of the circumstances that might be helpful. I think we'd

be derelict

> in our responsibility if we ignored this. "

>

> Already, Dr. Calne believes he may have one intriguing clue. The

production

> studio, a largely underground structure in downtown Vancouver, is

heavily

> insulated because of the need to block out sound.

>

> " Obviously the air circulation is very tight, " Dr. Calne said. " And

so if

> there was anything in the environment, there may have been a bigger

risk of

> that being recirculated to an extent that might amplify risk. "

>

>

>

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