Only last year, at the age of 47, Richard
Weeden was trapped in a body that could hardly function. Fourteen
years earlier, he had been diagnosed with Parkinson's disease, a
condition affecting areas of the brain that control movement.
For some time, his symptoms of trembling
hands, rigid muscles and slowness of motion had been controlled
by medication. But in 1989, he began to experience a common side
effect of the drugs, uncontrollable movements called dyskinesias.
When he walked, his legs would jerk up and forward, in a bizarre
parody of a military goose step, making it impossible to walk in
a straight line. Even at rest, his legs constantly writhed and twisted.
Without the medication, Weeden was
practically frozen. His hands trembled constantly, and any attempt
to move was excruciatingly slow. His balance was so poor that he
risked falling whenever he tried to walk. Sometimes he had to move
around his home on his hands and knees. Finally, he could not even
get out of bed. His wife Betty, a registered nurse, thought she
would have to hire an attendant to stay with him during the day
while she worked.
'Before Rick got Parkinson's, he was
very physically active,' she says. 'We waterskied, we sailed; he
loved to do carpentry work around the house. Then slowly, inexorably,
he lost everything.'
John Growdon, MD, the MGH neurologist
who oversaw Weeden's care, says: 'We were very frustrated with all
our efforts to help him with medication. He had only a few minutes
every day when he could function at all.'
Today, Weeden's situation has profoundly
changed. He walks more than five miles a day. He is able to care
for himself and work around the house. While he is not free of Parkinson's
symptoms, he is once again an active participant in the lives of
his family and friends.
Richard and Betty Weeden
an Obsolete Operation
This transformation is the result
of an operation performed at the MGH, an operation that resulted
from a collaboration between an MGH neurosurgeon and a Finnish surgeon
who is leading the revival of a procedure thought obsolete decades
The operation, called a pallidotomy,
was one of several used during the 1950s and '60s to treat Parkinson's.
All these (procedures involved making small lesions (areas of damaged
tissue) in parts of the brain involved with motion control. In the
pallidotomy, the lesion is placed in an area called the globus pallidus.
Through a process that is still not well understood, the operation
successfully relieves symptoms for many patients (see sidebar).
When medications that produced similar effects without surgery became
available in the late '60s, the pallidotomy was abandoned.
Neither drugs nor operations can cure
Parkinson's. Eventually patients need higher doses of medication
to control their symptoms. Like Richard Weeden, many develop drug-induced
dyskinesias and find themselves existing on a precarious edge: motionless
without medication, moving uncontrollably with medication.
For the most severely affected patients,
surgeons have continued to offer another operation called a thalamotomy.
But while that procedure does control tremor and muscle rigidity,
it can cause the third major symptom, slowness of movement, to get
Because of this limitation of the
thalamotomy, Lauri Laitinen, MD, PhD, a Finnish neurosurgeon working
in Sweden, began once more doing pallidotomies in patients with
the most severe symptoms. In 1992, he published results showing
dramatic results for most patients, sparking interest among many
neurosurgeons, including G. Rees Cosgrove,
MD , who was about to join the MGH staff.
Dr. Rees Cosgrove
News about Laitinen's success also
spread through support and information networks set up by Parkinson's
patients and their families. The Weedens heard about the operation
and traveled for an evaluation from their home in Portsmouth, R.I.,
to a New York teaching hospital that planned to offer the procedure.
A lack of coordination among the staff
of the New York center meant that the Weedens had to make several
trips to the city and back. When asked to make appointments with
several additional doctors, again requiring separate trips, they
decided not to return to New York.
Frustrated, the Weedens began to consider
traveling to Sweden. At the suggestion of Growdon, they also spoke
with Cosgrove, who had joined the MGH staff. While he had experience
with the thalomotomy operation, Cosgrove had not yet done any pallidotomies,
so he concurred that Weeden's best chance was to seek care from
Weeden was accepted as a patient by
Laitinen, and in what was to prove a lucky coincidence, his operation
was scheduled at the time when Cosgrove was in Sweden to learn the
procedure. Cosgrove observed his treatment and offered to provide
followup care back in the United States.
Unfortunately, the results of the
first operation in June 1993 were disappointing. Weeden experienced
some relief from the Parkinson's symptoms, but it lasted only a
few days. Laitinen recommended allowing time for his brain to heal
from the operation before deciding on a further course of action.
MRI examinations taken at the MGH
several weeks after Weeden's return pointed to a possible explanation
of the problem. 'From what I'd learned about the ideal placement
of the lesion, it looked like the position within the globus pallidus
was not quite right,' Cosgrove says.
Laitinen agreed with the assessment
and accepted an invitation to come to Boston and observe a second
procedure earlier this year. The two surgeons independently reviewed
detailed preoperative imaging studies, and both agreed on the specific
location for the new lesion.
In the operating room, Cosgrove made
a tiny hole in the skull and inserted a needle-like probe that carries
an electrical current into the brain. The electrode is insulated
to allow current to pass out at the tip only. Depending on how much
current is applied, the electrode can stimulate, inactivate or destroy
the cells it touches. In Weeden's case, the probe was passed into
the globus pallidus on the left side of the brain, which affects
movement on the right side of the body.
Because no pain-sensing nerves are
located inside the brain, patients remain awake during the operation,
under local anesthesia. With the help of the patient, the surgeon
can further confirm the target for the operation and avoid damaging
crucial nearby structures. 'We talk to the patients during the procedure
and do a number of intraoperative tests,' Cosgrove explains. 'By
passing a small current through the brain, we can see improvement
in function right away.'
When the electrode is in the right
spot, a stronger current is applied that destroys cells in a small
area. Weeden recalls the moment: 'Dr. Cosgrove was doing the operation,
and Dr. Laitinen was asking me to move my right leg to see if the
probe was in the right place. They agreed that the position was
right, and Dr. Laitinen said to me, 'You're not going to have any
trouble with that leg again.' I was skeptical, but since then it
has been rock steady. I haven't had any dyskinesia in that leg at
Weeden's entire right side, which
was most seriously affected before the operation, has improved remarkably,
and his sense of balance also has returned. Although he still takes
medication to con-
trol symptoms on the left side and
has some dyskinesia in that leg, he and his wife are delighted with
his restored mobility.
'There are so many things you take
for granted when you're healthy. I couldn't even roll myself over
in bed at night, much less get up to go to the bathroom,' he says.
'Now I take care of the house myself and make the meals. I can get
out for a walk every day. To me, that's just wonderful.'
The pallidotomy is not a cure for
Parkinson's; symptoms can eventually return as the degenerative
process that underlies the disease continues. But the operation
will most likely give Weeden several additional years of functional
mobility. And if symptoms on his left side worsen significantly,
the operation can be repeated on the untreated right side of the
brain for a few more years of relief.
'Rick's improvement has been like
a miracle, and we're so grateful to Dr. Cosgrove, Dr. Laitinen,
and everybody at the MGH,' Betty Weeden says. 'The nurses who cared
for Rick were just fantastic. Before this operation I felt like
I didn't have my husband any more; I was a nurse taking care of
a patient. Now I have my husband back.'
Trying to Restore the Balance
Parkinson's disease is caused by the
death of certain brain cells secreting a chemical called dopamine,
one of several neurotransmitters that carry signals between brain
cells. Normally, dopamine operates in a delicate balance with other
neurotransmitters to help coordinate the millions of nerve and muscle
cells involved in movement.
Without enough dopamine, this balance
is upset. People begin to exhibit the typical symptoms of Parkinson's
disease -- a trembling of the hands or head, increasing rigidity
of muscles, and a slowness of motion. The disease typically appears
in older people, with most being diagnosed in their 50s or 60s,
and usually continues to worsen throughout life. Some patients,
like Richard Weeden, develop symptoms much earlier.
John Penney, MD, an MGH neurologist,
evaluates Parkinson's patients for surgery. He notes that several
conditions can produce symptoms of the disease -- including infections
of the brain, certain drugs, and brain injury (as in the case of
boxer Muhammad Ali). But the ultimate cause of true Parkinson's
disease is still a mystery.
For decades, neurosurgeons tried to
relieve the involuntary movements of conditions like Parkinson's
by operating on parts of the brain involved with motion control.
As more was learned about brain anatomy, attention was directed
toward a cluster of deep-brain structures, including the basal ganglia,
the thalamus, and the globus pallidus. These form a 'circuit' that
transfers motion-related signals from the brain to the spinal cord
and thus to the rest of the body.
In the late 1940s, introduction of
a technique called stereotactic surgery made operating on the brain
much safer. Stereotaxis uses a metal frame to hold a patient's head
absolutely still during imaging studies and the operation. Measurements
can be taken to precisely locate structures within the brain, and
delicate instruments inserted through a tiny hole in the skull to
remove or destroy tissue.
With less risk of damaging healthy
brain tissue, surgeons used stereotactic surgery to control the
symptoms of Parkinson's by creating small lesions within these brain
structures. While such operations were used to treat advanced Parkinson's
symptoms during the 1950s and '60s, they were largely abandoned
when drug treatment became available. One of the operations, the
thalamotomy, did continue to be offered, and now pallidotomy, creation
of a lesion in the globus pallidus, is being revived for selected
'This operation is not for everyone,'
says John Growdon, MD, MGH neurologist. 'Most patients are treated
successfully with medication. But each patient is unique, and a
few, like Mr. Weeden, develop exaggerated responses to the drugs
that can be just as disabling as the illness. Without this operation,
there isn't much else we can offer such seriously affected patients.'
Why making a lesion in the brain can
relieve Parkinson's symptoms is a mystery. Some evidence indicates
that part of the globus pallidus is overactive in the disease. The
lesion may suppress that area and partially restore the lost balance
between neurotransmitter systems. G. Rees Cosgrove, MD, the MGH
neurosurgeon who performs pallidotomies, has now done a total of
eight operations, seven of them at the MGH, which currently is the
only hospital in New England offering the procedure.