Parkinson's Disease: A Complete Guide for Patients and Familiesby Lisa M. Shulman, Anthony E. Lang
In recent years, new treatments have vastly improved the lives of people with Parkinson's disease by substantially reducing symptoms and significantly delaying the onset of disabilities. Parkinson's Disease: A Complete Guide for Patients and Families offers information to help maintain quality of life while living with Parkinson's disease. The book helps readers… See more details below
In recent years, new treatments have vastly improved the lives of people with Parkinson's disease by substantially reducing symptoms and significantly delaying the onset of disabilities. Parkinson's Disease: A Complete Guide for Patients and Families offers information to help maintain quality of life while living with Parkinson's disease. The book helps readers understand the disorder and offers guidelines for working in partnership with a physician to develop effective coping skills and make educated choices when considering the array of treatment options.
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What are the symptoms of Parkinson's disease?
What causes these symptoms?
What is the difference between Parkinson's disease and parkinsonism?
When should I tell other people that I have Parkinson's?
Whenever a diagnosis of Parkinson's disease is made, patients and families naturally ask, "What is Parkinson's disease?" As doctors treating people with Parkinson's, of course, we discuss this question with many patients. But weand theyare aware that the answer is both straightforward and elusive.
Parkinson's disease is a degenerative neurologic disease. Degenerative means "declining in quality"thus, the disease increases in severity over time; neurologic refers to the nervous system. One could therefore say that Parkinson's disease is a disease of the nervous system that gets worse over time.
We also describe Parkinson's disease as a chronic, progressive neurologic disease. Chronic means "of long duration"; progressive means "proceeding in steps" or "advancing." Parkinson's disease does not go away, and it gradually gets worse.
Parkinson's disease is named after the English physician James Parkinson, who first described the illness. His original paper describing this disorder, published in 1817, was entitled "Essay on the Shaking Palsy," and to this day Parkinson's disease is still sometimes called the"shakingpalsy." Another name for this illness is paralysis agitans, which is simply the Latin translation of "shaking palsy." The names Parkinson's disease, shaking palsy, and paralysis agitans all refer to the same illness.
There is some fairly good news. True Parkinson's disease progresses slowly. Even after the symptoms have become serious enough and clear enough to allow a definitive diagnosis, it is usually years, maybe a decade or more, before a person suffers from a serious disability. Further, treatments are available that can relieve symptoms, so that years, sometimes a decade or more, can go by before symptoms have a significant impact on a person's quality of life.
In people with Parkinson's disease, specific groups of brain cells called neurons are slowly and progressively injured, then selectively degenerate or die. This process causes the typical symptoms of Parkinson's disease, which doctors call "characteristic symptoms" because they are the major features of Parkinson's. (In this book, when we say symptoms are "characteristic" of or "characterize" a disorder, we mean they are typical of that particular disorder and distinguish it from others.)
These are the characteristic symptoms of Parkinson's disease: People who have Parkinson's may tremble involuntarily. They find their muscles become rigid and stiff, and they lose their ability to make rapid, spontaneous movements. They walk in a recognizable manner, with a typical gait in which the body is bent or flexed, and they may have difficulty maintaining their balance. The characteristic symptoms of moderate Parkinson's disease can be remembered with the acronym TRAP: T is for tremor and R for rigidity. A is for akinesia (meaning, literally, "lack of movement"), referring to the loss of spontaneous or voluntary movement and loss of fluid motion (the slowing down, rather than complete loss, of movement is called bradykinesia). P is for postural instability, which involves difficulties with balance and the risk of falling (Table 1.1). Parkinson's has neither a cure nor any treatment to slow down its progression.
Furthermore, the signs and symptoms of early Parkinson's are only subtly different from those of other diseases, some more serious and some less serious than Parkinson's. The similarities among these diseases can make diagnosis difficult, and as frustrating as it may be for a person with Parkinson's symptoms, often the only way to identify Parkinson's disease for sure is to wait and see (see Chapter 8).
What Happens in Parkinson's Disease?
In Parkinson's disease, neurons (nerve cells) of the brain area known as the substantia nigra (Latin for "black substance") are primarily affected (Figure 1.1). When neurons in the substantia nigra degenerate, the brain's ability to generate body movements is disrupted and this disruption produces signs and symptoms characteristic of Parkinson's diseasetremor; rigidity; akinesia (lack of movement or loss of spontaneous movement) and bradykinesia (slowness of movement); and problems with walking and posture.
The symptoms of any brain disease are determined in part by the location of the neurons that degenerate. For example, Alzheimer's disease involves the degeneration of neurons of the cerebral cortex and results in memory loss and mental deterioration. In amyotrophic lateral sclerosis (ALS, or Lou Gehrig's disease), the selective deaths of motor neurons in the spinal cord and brain cause profound motor weakness. Again, in Parkinson's disease, the affected neurons are located in the substantia nigra, an area of the brain that is important for control and regulation of motor activity (movement).
What Causes the Symptoms?
The substantia nigra is a very small area located deep within the brain. There is one substantia nigra on the right side of the brain and one on the left, but for ease of discussion, the medical literature refers to them as if they were a single structure. The symptoms of Parkinson's disease do not become noticeable until about 80 percent of the cells of the substantia nigra have died, because the human nervous system has multiple safety factors and redundancies built into it. For a long time, these safety factors are able to take over the activities of the dying cells.
In autopsies of persons with Parkinson's disease, the brain appears to be relatively normal except that the substantia nigra has lost its usual black pigment (Figure 1.2). Under the microscope we can see substantially fewer cells in this substantia nigra than in that of healthy brains, and the remaining cells often show signs of abnormality. One hallmark of Parkinson's disease is the presence of small bodies known as Lewy bodies within the remaining substantia nigra cells.
The substantia nigra accounts for an extremely small percentage of the brain's weight, but because of its important electrochemical connections with motor centers (brain centers that control movement), it is a vital component in how we move. Specifically, a series of complicated electrical and chemical events within the brain transmits information from neuron to neuron. The chemicals that brain cells use to communicate with one another are called neurotransmitters or, more generally, neurochemicals. The specific neurotransmitter produced and used by the substantia nigra is dopamine. When the cells of the substantia nigra degenerate and die, dopamine is lost and dopamine-relayed messages to other motor centers cannot go through. This is the primary cause of the motor symptoms in Parkinson's disease.
There is more. While the loss of dopamine-producing cells is the primary, neurochemical disturbance in Parkinson's, the neurochemical disturbances are not limited to the cells of the substantia nigra and to the loss of dopamine. Other small nuclear centers within the brain (e.g., the regions called the dorsal motor nucleus of the vagus and the locus ceruleus) also are affected by the degeneration. In Parkinson's disease, as the concentrations of dopamine in the brain decline, so do concentrations of other neurotransmitters such as norepinephrine and serotonin, although changes in these other neurotransmitters are not nearly as significant as the loss of dopamine. These neurotransmitter and cell changes are spread widely throughout the brain, which may help explain why dopamine replacement does not correct all the problems caused by Parkinson's. In short, Parkinson's disease is not just a dopamine deficiency state.
Although we have some understanding that neurochemical disturbance causes the symptoms of Parkinson's, we still do not know what causes the neurodegeneration, although extensive scientific research has been conducted in this area. For this reason, the disease is sometimes termed idiopathic (cause unknown) Parkinson's disease. In Chapter 2 we look at what the current research indicates, and in Chapter 17 we investigate some leads that may shed light on the causes.
Because the brain's neurotransmittersespecially dopamineare so important to the central nervous system's control of the muscles, when these neurotransmitters are lost, the muscles act strangely. The central mechanism that controls muscle tone is altered. The muscles may tighten up at inappropriate timesrapid tightening and releasing of muscles produces a tremor. Sometimes the muscles tighten up and become stiff and rigid. With inadequate communication between the brain and the muscles, movement also becomes slow: muscles cannot make quick, fluid, spontaneous movements. The central mechanism that controls muscle tone does not function adequately for the delicate interplay of muscles required to help us stand, walk, and balance. In addition, because Parkinson's disease also affects the autonomic nervous system (the largely unconscious system that controls our body temperature, digestive system, sexual function, and bladder control, among other functions), these systems may also act oddly.
In true Parkinson's disease, the first symptoms differ only slightly from the normal state and progress slowly, perhaps over decades. Different people have different combinations of symptoms.
Any person who has the signs and symptoms characteristic of Parkinson's disease (tremor, rigidity, slowness of movement or loss of spontaneous movement, and postural impairment) is said to have parkinsonism, but not every person with parkinsonism has Parkinson's disease. Parkinsonism has many possible causes, and Parkinson's disease is only one of the possibilities. For example, parkinsonism may be the result of a stroke or a side effect of certain medications. Many other types of neurodegenerative disorders result in parkinsonism, although Parkinson's disease is the most common of these. In many cases the parkinsonism found in these other disorders is due to damage to the substantia nigra, and more often than not the damage extends to other brain areas as well. People with parkinsonism may have symptoms of impaired movement, thinking, behavior, and other body functions (such as blood pressure and sexual, bladder, and bowel function) that people with true Parkinson's are less likely to develop.
Even considering such differences, Parkinson's disease can be very difficult to distinguish from other forms of parkinsonism. Patients and their families need to understand parkinsonism, because some 20 to 25 percent of people diagnosed with Parkinson's disease will eventually be discovered to have some other form of parkinsonism. Parkinsonism may look like Parkinson's disease, but over time it does not act like it. Differences that were subtle at the beginning of a disorder often become more pronounced as it progresses. For people with parkinsonism, the symptoms may become more disabling more quickly or may progress more slowly than in Parkinson's disease. Parkinsonism symptoms may or may not respond to the medications used to treat Parkinson's disease.
A number of other disorders that include involuntary tremor are not, strictly speaking, a type of parkinsonism but can be mistaken for Parkinson's disease.
If you have been diagnosed with Parkinson's but observe that your symptoms are not characteristic of Parkinson's disease as described in this book, you may have another form of parkinsonism or a different type of disease altogether (Table 1.2) (see also Chapters 8, 9, and 10).
What Will Happen to Me If I Have Parkinson's?
Because Parkinson's disease is a progressive disorder, we can generally expect that each year the signs and symptoms of the disease will become more pronounced. No one, not a physician or anyone else, can accurately predict how, or how quickly, the disease will progress in a specific individual. There simply is no reliable way to evaluate the degree of cell loss in the substantia nigra, no laboratory test or widely available imaging procedure that can tell us how much cell loss has occurred or how fast it is progressing.
We can say that Parkinson's is not the kind of disease in which, within a twelve-month period, someone who is able to walk and function independently suddenly finds herself or himself incapacitated and wheelchair-bound. In the average patient, the disorder is very slowly and gradually progressive over years, with relatively mild and subtle changes occurring in the first months to years of the illness.
Although we can't predict how the disease will progress in a specific individual, we do try to answer the questions patients naturally have about the course of their disease. Why? Because people need to know how long they can expect to remain employed or fully self-sufficient. They need to know how their disease will progress for financial, employment, and social planning reasons.
Disease progression varies greatly from one person to another. Some patients have a relatively rapid progression, beginning to experience significant physical disability within five years; others do not reach this state of disability for fifteen years. Sometimes these different disease courses are separated into "benign" and "malignant" forms. In our experience, most so-called malignant cases of Parkinson's disease have proved to be other diseases, which in their early stages were mimicking true Parkinson's disease (see Chapters 9 and 10).
A person's history with the disease is the best guide to the character of his or her illness in the future. In other words, a person's disease usually progresses at about the same rate that he or she has already experienced. This means that predictions are particularly difficult for people newly diagnosed with Parkinson's disease, because we have no information about how their disease has progressed so far. General information may be the best we can offer. In general, then, we say that when Parkinson's disease is diagnosed early, and when medications are used judiciously, people may experience five to ten years of motor symptoms that do not substantially interfere with their quality of life. Again, Parkinson's disease is not the kind of disease in which rapid deterioration occurs over a few months.
Although we do not yet have treatments capable of slowing or arresting the progression of the illness, current treatments can very effectively relieve the symptoms, especially in the early years. Many persons who are adequately treated notice very little or no progression of symptoms over the first few years. With time, a person's degree of motor disability does tend to increase, however, and after five to ten years of illness the symptoms will disrupt daily life. At this point, medications are needed in higher doses and must be monitored and adjusted more frequently.
Only in the last thirty years have dramatic breakthroughs been made in the management of Parkinson's disease. As noted above, current treatment can significantly relieve people's symptoms and markedly improve their quality of life. Unfortunately, there is as yet no cure. (Treatment is discussed in more detail in Chapters 12 through 15.)
The first stage of treatment for Parkinson's is an accurate diagnosis. This is tricky, as we have noted, particularly early in the disorder when distinguishing Parkinson's disease from other diseases with similar symptoms is particularly difficult. It may be helpful to see a neurologist who is experienced with what are called movement disorders. A movement disorder specialist has expertise in diagnosing and treating Parkinson's disease and related disorders. A visit to a movement disorder center may also be useful. Most such centers are connected with a department of neurology at a medical school, although some are freestanding clinics. The centers have access to appropriate rehabilitation facilities and are usually involved in research studies.
We believe patients' involvement in research studies is a good idea, for several reasons. First, treatment of Parkinson's disease can be improved when people with Parkinson's consent to participate in programs that test drugs, so there is an altruistic motive for some people. Second, in clinical research trials, new treatments are tested on patients in carefully planned studies designed or monitored by physicians and statisticians, and people in these trials might have access to helpful drugs years before the drugs are available to others. Finally, people who participate in clinical trials tend to do better than people who do not, even when the people in trials are receiving a placebo rather than a drug. This is because participants in clinical trials receive intense medical attention and monitoring; they are working with people who are especially interested in their disease; they are actively participating in controlling their disease; and they feel better. (See Chapter 17 for more information about medical research and clinical trials.)
You can find the names of movement disorder specialists by consulting Parkinson's disease patient organizations or by calling teaching hospitals associated with medical schools. Many of the Parkinson's disease Web sites for laypersons have lists of neurologists specializing in movement disorders. You can also write to the Movement Disorders Society or the American Academy of Neurology and obtain the names of movement disorder specialists in your geographic region.
Even if you live some distance from a specialist neurologist or specialist center, you may want to consider traveling to a specialist or center on one or more occasions for a confirming diagnosis, a second opinion, or a treatment plan. Often a specialist who is consulted in this way will remain available to your internist or your family doctor for consultation by telephone. For example, people living in, say, Montana, might, if they can afford it, go to the Mayo Clinic to visit a specialist. On returning home, care can generally be coordinated with a local neurologist or family doctor. Sometimes patients who live a long way from a specialist or center monitor their own symptoms and, rather than playing phone tag, fax their questions to the specialist's office. The specialist then faxes back answers and recommendations. This is not the best possible system, but sometimes it is the best that can be managed.
Patients who belong to a health maintenance organization (HMO) usually are obliged to use the HMO's doctors unless they get a special referral for someone outside the organization. The HMO may have its own neurologist. If you are in an HMO that does not have a neurologist, lobby firmly for a special referral.
When Should I Tell My Family, Friends
As we note later in this book, family membersspouses, children, siblingsare often the first to notice symptoms in the person with Parkinson's disease. They may notice a change in the way a person walks or stands or in the person's expression. But even if family members haven't yet noticed any symptoms in the person with Parkinson's, they will notice them as time goes on. If you have been diagnosed with Parkinson's, rather than letting people close to you wonder and worry about what's wrong, it is generally best to tell them about the disease and that you are being treated for it. You can also assure them it is not a contagious disease and there is very little evidence that it can be passed from one generation to another.
Family members will share your concerns, of course, perhaps especially about the uncertainty of the futurethe progression of the symptoms. But sharing your concerns as a family is almost always better than one person "braving it out" alone. And this way, too, the family can make plans for the future, perhaps taking that cross-country trip now rather than putting it off for years, as well as putting some savings away.
You would not be able to hide the symptoms of Parkinson's disease from your family indefinitely, and for this and these other reasons, our advice is to tell your family. Choose a place and time when you and they are comfortable, not stressed, and make sure you have lots of time to talk and share your feelings. If there are problems or tensions in your family that might make the telling difficult or even traumatic, you might want to enlist the help of a professional counselor or social worker, who can either advise you on how to break the news or be with you and your family during this time.
Deciding on the best time to tell an employer and colleagues about a Parkinson's diagnosis is often difficult. A person carrying a full load of responsibilities at work is naturally concerned that people will become uncertain about his or her ability to function if they learn about the diagnosis of a chronic illness. And people do sometimes react this way. Nonetheless, if you are in this situation, it is not a good idea to suppress information about your illness for too long. If your co-workers do not know about the disease, they might get some very wrong ideas: they might think the tremor means nervousness or anxiety, the masked face means boredom, and the soft voice means a lack of conviction.
Parkinson's disease is a difficult disease to hide, anyway. People with a tremor would need to make up excuses about why their hand shakes. Or, if they tried to hide the tremor, they might become quite anxious, which makes the tremor worse. Most people decide it is just better to say, "I have Parkinson's."
Although Parkinson's is a fairly common disease, what most people "know" about it is often based on their experience with a relative or friend from some years ago. They probably aren't aware that the new medications are much better at managing the symptoms or that people with the disease can continue to function well. So, once you make the decision to share this news with co-workers, keep in mind that they will react better to the news if you educate them a bit about how the disease doesand does notaffect you.
There are other considerations. The federal Americans with Disabilities Act (ADA), signed into law in 1990, states that in a company with more than fifteen employees, the company is required to make "reasonable accommodations" in the workplace so that a person with a disability can continue to work. The ADA bans discrimination in employment against qualified persons with disabilities.
If oral presentations are a large part of your job, you may have difficulty if your voice becomes softer and more monotonous. Microphones and speaker systems can be used to make the presentations audible even to a large audience, however, so that your work can go on.
If you must have steady hands in your job, you may find your colleagues are alarmed by the tremor, even though it disappears when you are moving. You may need to explain about this aspect of tremor and demonstrate your abilities. Eventually, if tremor does become a problem, you may need to take on different tasks and responsibilities and give up tasks requiring a steady hand. Also, if stress and emotional intensity make the tremor worse, you may need to take care to avoid stressful situations.
The mood changes that sometimes appear early in Parkinson's may pose a problem for some people in a work situation. Someone with depression may find it very difficult to work effectively, for example; medical treatment of the depression generally helps with both the depression and effectiveness at work. A person who becomes unusually anxious in situations that previously would not have caused such distress may find medications can help relieve the anxiety (see Chapter 13). For many people, continuing to work and be productive is an important factor in adjusting to the diagnosis of Parkinson's disease.
BY NOW IT IS CLEAR WHY PATIENTS AND DOCTORS alike understand that defining Parkinson's disease is both straightforward and elusive. The straightforward part is that we can describe Parkinson's disease as a specific neurologic disorder in which the destruction of specific centers within the brain disrupts people's control of movement. The disorder primarily involves a chemical deficiency of the neurotransmitter dopamine, and (as we'll discuss in Chapter 12) the replacement of dopamine significantly relieves the characteristic disease features of tremor, rigidity, slowness of movement, and difficulty with balance and posture.
What remains elusive is a precise definition and description of Parkinson's, as well as any understanding of its fundamental cause or causes. And progress of the disease varies significantly among people with the same signs and symptoms, and perhaps the same diagnosis, and we don't know why.
Here is the closest we can come to defining Parkinson's disease at this time: It is a rather slowly progressive neurodegenerative disease that has the characteristic symptoms of tremor, rigidity, slowness of movement, and gait and balance problems. These symptoms can be relieved by administering antiparkinson medications. The degeneration in Parkinson's occurs in the substantia nigra and the regions of the brain's motor control system downstream from the substantia nigra. A number of other diseases have some of these characteristics, but only Parkinson's disease has all of them. As we learn more about the disease we hope to understand not only what causes it but how it can be treated more effectively and, someday, how it can be prevented.
In the next chapter we look at who gets Parkinson's disease, then we turn, in Chapter 3, to a description of the early symptoms.
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