It has been more than 20 years since I first learned that I had Parkinson's disease. I had complained for about six months of aching discomfort in my right arm, fairly typical of thoracic outlet syndrome, or so I believed. I was a vascular surgeon, operating in a hospital affiliated with Harvard Medical School. For me to have a stiff arm was a nuisance.
Wasting no time on the thoracic outlet, my consultant neurologist, gently and not without some sadness, examined me briefly and told me that I had Parkinson's disease. He explained the bare outlines of the disease, bringing me up to date on recent developments, but I heard little of the story he was telling.
I was devastated. As a physician, I had seen many patients suffer from this condition, which is rather common, as documented elsewhere in this issue of the Journal by Bennett and colleagues. But being a patient was different. Somehow, I had not considered that the grasp of this terrible disease might extend to include me. Would I be able to practice my profession? Could I support my family? Would I be physically active? And could I expect to have a normal life span?
It happened that at that time I was negotiating with a major hospital to become chief of its Department of Surgery. This was my career goal. Could one expect any medical school to select as its chief of surgery a nonoperating surgeon with a motor disorder? All these years, surgery had been my life, my identity. Could I find the same satisfaction in some other activity?
Parkinson's disease is a chronic, progressive disease, although its progress may be very slow. Recently, I reviewed hospital records in which I had written consultation notes when I was a surgical resident in the 1950s. Many of the passages I had written were micrographic and typical of Parkinson's disease. Thus, I may have had my illness for more than 20 years perhaps for as long as 40 years! A patient with newly developed Parkinson's disease who also has imagination will have sufficient time to explore alternative occupations, such as nonoperative medicine and teaching. Often, a patient with Parkinson's disease will have years in which to practice a second career.
Surgeons with Parkinson's disease must find new work. I stopped operating when my diagnosis became clear, long before I did any damage. It was, in my view, absolutely essential that I continue to be productive. I was fortunate to have had a long-time interest in research into problems of thrombosis. After the end of my residency I was able to establish a small research laboratory, supported by a grant from the National Institutes of Health (NIH) and local funds. It was not difficult to expand the activity of that lab in an era when the NIH was more generous. NIH funding as a basis for long-time support might be more difficult today. The academic life is only one example of the alternative careers a surgeon with Parkinson's disease might consider. It is an alternative that would not have been available to me had I been a full-time clinician performing surgery in the community.
The classic course of Parkinson's disease is one of inexorable progression to an end stage, but this may take many years. A large fraction of patients are aged or have other medical conditions and do not live long enough to experience severe disease.
A patient's attitude is often a good guide to his or her ability to cooperate with therapeutic efforts. A positive attitude is valuable, for one is faced with a chronic illness that can be frustrating. One of its most trying aspects is the extent to which it interferes with the trivial events of daily life. Nothing is easy in Parkinson's disease. There is no feature of any task that is not potentially out of control. A cuff link refuses to find its way into a tuxedo shirt cuff, my wife is out of town, and I miss the annual dinner. I am unable to stuff change from a $5 bill into my wallet, and the patrons in line behind the cash register fume. Bow ties won't tie and shoelaces won't lace. A cube of beef obstructs the glottis. In Parkinson's disease one must expect the unexpected.
Furthermore, even when an equilibrium is apparently achieved, it is not really secure. In the course of my illness, I have spilled a full tumbler of grapefruit juice over the head of a nurse in a hospital cafeteria, squirted a melted chocolate bar from my clenched fist onto patrons in the brightly lit front row of a balcony at Boston's First Night celebration, had an attack of athetoid and choreiform hyperkinesia during a Boston Symphony Orchestra concert, and fallen at home and about the city too many times to count. I have had two successful Heimlich maneuvers performed once by an old family friend who was a practicing veterinarian and once by my wife, who is a good observer.
Everything takes longer in Parkinson's disease, but eventually it does get done. This can be a source of great personal satisfaction, because it means that, given the time, one can live a near-normal life that is not housebound. Thankfully, I have been able to continue an active social schedule of concerts, plays, and travel as part of an attempt to keep Parkinson's disease in its place. Although I may have required assistance from a companion, I have seldom had to stay home.
My wife, family, friends, and a devoted secretary provide an essential support system for me. Our three sons first learned of my disorder from me during a wild sailing cruise in the Mediterranean; they could see that I was not yet over the hill. Professional colleagues remained close allies until late in my course, when I became sicker. Curiously, I found that some of them began to avoid me, I think because they were embarrassed by their inability to do anything to ameliorate my problem. I still count them among my friends. The people at Beth Israel Hospital and Harvard Medical School have been as solid as a rock. They are completely dependable.
I spent 12 years of this period on the staff of the New England Journal of Medicine, a joyous association and one that I cherished. I was deputy editor half-time, which enabled me to run my lab and keep my Harvard appointment as well a busy schedule.
About five years ago, my disease began to close in on me, becoming more aggressive and difficult to handle. I had increasing discomfort from hyperkinesia, my voice was almost inaudible, and periods when my feet felt frozen to the floor became commonplace. I lost the advantage I had previously enjoyed of a comfortable margin between an effective dose of medication and a dose with intolerable side effects. I had an "off" spell (a sudden loss of the effect of medication) in a telephone booth in Harvard Square. Adjustments in my dose of Sinemet seemed to make little difference. I found myself overwhelmed by my disease and unable to set it aside while I concentrated on the business of living. I gave up driving. I joined clinical trials of promising new drugs. My appetite failed. I lost 20 pounds. I was miserable.
While I was preoccupied with my own problem, the larger world of Parkinson's disease was experiencing its greatest excitement since Cotzias's discovery of the effect of levodopa: namely, the arrival of the operation of pallidotomy. The goal of this stereotactic neurosurgical procedure is to identify and destroy part of one of the basal ganglia, the globus pallidum. This structure secretes neurotransmitters in response to the depletion of dopamine, which results from degeneration of the substantia nigra. Secretion by the globus pallidum is believed to account for most of the symptoms and signs of Parkinson's disease, including tremor, muscular rigidity, and bradykinesia. The operation therefore affects the response to Parkinson's disease but not, it is thought, the course of the disease itself. During the operation the patient remains awake and reports the effects of stimulation of the optic tract and other vulnerable structures. The globus pallidum is located stereotactically and destroyed by heat. Neuron mapping by electrophysiologic techniques provides the surest protection of nearby structures, but some maintain that this very demanding part of the procedure is unnecessary. Comparative studies to resolve this dispute are in progress.
Hundreds of patients have had this operation performed, and as a group they have done extremely well. An air of a visit to Lourdes surrounds it, and there are waiting lists at institutions where the operation is performed. Although it does not work miracles, many people believe that the operation relieves most of the manifestations of the disease in most patients. The literature is very limited, often uncritical and inadequate, and the results vary in different institutions. There are complications. Nonetheless, the overriding impression is positive even enthusiastic as desperate patients have seized onto the operation, hoping for some relief.
On August 9, 1995, I underwent a pallidotomy with electrophysiologic monitoring. By the end of the operation, most of my motor symptoms were gone. On September 1, I awakened at dawn, hopped out of bed, and drove myself to my favorite fishing spot. Independent at last! A personal victory.
Edwin W. Salzman, M.D.
Beth Israel Hospital
Boston, MA 02215
The New England Journal of Medicine is owned, published, and copyrighted © 1996 Massachusetts Medical Society. All rights reserved.To the Editor: Dr. Salzman gives a moving account (Jan. 11 issue)1 of his 20 years as a patient with progressive Parkinson's disease. His description of the various phases of the illness and his struggle to stay in control present a fine model for other patients to discover ways to accommodate themselves to the disease without surrendering their dignity.
We are concerned that his overly generous praise of pallidotomy may mislead readers who uncritically accept his statement that pallidotomy "relieves most of the manifestations of the disease in most patients." Pallidotomy effectively reduces levodopa-related dyskinesias and limb rigidity on the side contralateral to the lesion, and to a lesser extent it can lessen tremor and improve walking. Unfortunately, many of the medically intractable symptoms, such as postural instability, falling, muffled speech, dysphagia, and cognitive decline, do not improve. Even when the benefit of surgery is dramatic, the duration of the response is still unknown.
It is gratifying that pallidotomy has become a serious treatment option for well-selected patients with Parkinson's disease. However, it is a mistake to make unqualified generalizations about all patients when the evidence at this time permits only cautious optimism for some.
Howard I. Hurtig, M.D.
Matthew Stern, M.D.
Amy Colcher, M.D.
Philadelphia, PA 19146
Steven R. Simon, M.D.
University of Hawaii
Honolulu, HI 96813-2427
J. Richard Baringer, M.D.
University of Utah
Salt Lake City, UT 84132
Pallidotomy has become a very controversial treatment for Parkinson's disease.1,2 Some physicians have grown skeptical of the results because of sensationalized coverage by the media and the recurrence of symptoms in a few patients weeks after surgery. The reason for the transient benefits is not clear, but it is probably at least one of the following: the lesion was in the wrong place; the lesion was not large enough; or the patient did not have idiopathic Parkinson's disease.
We recently studied the time course of radiofrequency lesions in the brain with serial magnetic resonance imaging immediately after surgery.3 The edema related to these lesions appeared within 20 minutes of the placement of the electrode.4 Therefore, patients could experience the benefits of the decreased ventroposterior pallidal activity due to the formation of edema, although the lesion was not quite in the desired site. Moreover, long-term follow-up with magnetic resonance imaging shows that in some patients the lesion is completely healed and almost imperceptible (Hariz M: personal communication). We also evaluated a number of patients with poor outcomes who underwent pallidotomy at other centers. The majority of these patients did not have idiopathic Parkinson's disease but had progressive supranuclear palsy, multiple-system atrophy, or vascular parkinsonism. These disorders do not generally respond favorably to stereotactic pallidotomy.
To avoid this pitfall, we adopted a plan involving several steps. First, each patient is evaluated by a neurosurgeon and a neurologist specializing in movement disorders. Second, the patient is monitored closely by a neurologist during the procedure. Third, 2-Hz stimulation is used to detect the internal capsula or optic tract. Stimulation with 50 Hz can increase outflow from the globus pallidus internum to the thalamus, and therefore an increase in tone suggests proper placement of the electrode. A gradual increase in the stimulation current provides a physiologic measure of the distance of the probe from structures to be avoided. Fourth, the lesions are created in two steps. The temperature is first increased to 45°C during the time needed for a complete neurologic examination. Then, if a positive response is observed and no undesirable effects are detected, the temperature is increased to 80°C and held constant for one minute.5 The importance of electrophysiologic studies and neurologic examination during surgery cannot be overemphasized. This serves to advise patients to avoid radiosurgical ("gamma knife") pallidotomy, because electrophysiologic and neurologic monitoring are impossible with this technique.
Antonio A.F. De Salles, M.D., Ph.D.
Jeff Bronstein, M.D.
Donna Masterman, M.D.
University of California, Los Angeles
Los Angeles, CA 90024-6975
Not only the writing, but also the courage and grace under stress, show through very clearly.
David L. Sagman, M.D.
New York, NY 10034-1159
Harold F. Dvorak, M.D.
Harvard Medical School
Boston, MA 02215
To the Editor: I welcome the advice of Hurtig et al. and De Salles et al. that caution should be used in deciding to perform a pallidotomy. Clearly, this is not an operation suited to every patient. The need for accurate diagnosis is well recognized; as the writers warn, a patient who does not have Parkinson's disease does not appear to be a good candidate. Their statement that pallidotomy is primarily effective in treating dyskinesia and rigidity is misleading, however, since considerable evidence, both old and new, indicates that pallidotomy can substantially reduce tremor and bradykinesia and improve gait and balance as well as posture. It is now well known that much of the morbidity associated with the disease results from its treatment. Pallidotomy relieves these symptoms.
My report was not, of course, an analysis of pallidotomy, but rather a description of the effect of a chronic, debilitating disease on the life of a physician.
I am grateful to Drs. Simon, Baringer, Sagman, and Dvorak for their kind remarks, and especially to Dr. Dvorak for his fish question. Unfortunately, I did not catch any fish. After all, no medicine is perfect.
Edwin W. Salzman, M.D.
Beth Israel Hospital
Boston, MA 02215