What is Parkinsons?
- Ms. Susan Reese, Director of the Young-Onset Parkinson's Disease Information & Referral Center of the American Parkinson Disease Association.

Parkinson's disease is often thought of as an "old people's" disease; however, approximately 10% of the population diagnosed with Parkinson's disease is under the age of 40. These patients and their families must live for many years with the medical and emotional challenges that the disease presents. Knowledge of the medical management issues, psychosocial concerns, and resources available for the young-onset Parkinson's patient will assist the health care provider in planning for long-term control of symptoms and an optimum quality of life for the patient.

(Home Health Care Consultant 1999;6[4];26-29)

In 1817, Dr. James Parkinson, in his Essay on the Shaking Palsy,1 first described a puzzling set of symptoms that he found in some of his older patients. The condition he described, dominated by "a pronessto trembling and an increasing sense of weakness," became known as Parkinson's disease, named after the physician who first wrote about it in the medical literature. The disease is sometimes known as paralysis agitans, which translated from Latin means "shaking palsy." It remains the official name for the disease in the World Health Organization's International Statistical Classification of Disease.2

It is, therefore, not surprising that even today the mention of Parkinson's disease for most people conjures up images of older adults, shaking and stooped with a shuffling gait. Although the average age of diagnosed onset for Parkinson's disease is in the early 60s, approximately 10% of the 1.5 million people with the disease are thought to be below the age of 40.3 However, among those patients diagnosed in midlife, it is not uncommon to hear reports of a long history of puzzling aches, stiffness, and clumsiness that may actually have been the early signs of Parkinson's disease.

Often the younger patient will report a lengthy, circuitous route to the eventual diagnosis of Parkinson's disease, which may have included consultation or treatment from a variety of specialists such as rheumatologists, orthopedic surgeons, and psychiatrists.

Because Parkinson's disease is still often overlooked as a diagnosis in younger patients, it is thought that the number of cases occurring in individuals below the age of 40 may actually be much higher than the estimated 10% of the population with the disease. With the advent of new drugs that may have neuroprotective benefit and slow the progression of the disease, it is crucial that an early diagnosis be made, particularly in the younger patient who will have many more years to deal with the disease.

WHAT IS YOUNG-ONSET PARKINSON'S DISEASE?

When Parkinson's disease is seen in individuals under the age of 40, it is called "young-onset Parkinson's disease." In rare instances, Parkinson's-like symptoms can appear in children and teenagers. This form of the disorder, called "juvenile Parkinsonism," is viewed as a distinct disorder, which often is genetically determined. Juvenile Parkinsonismusually has a different course from typical later-life Parkinson's disease.

Contrary to Dr. Parkinson's description and the Latin name, Parkinson's disease does not necessarily include shaking or tremor. In fact, tremor is reported with somewhat less frequency in younger patients than in those diagnosed in their sixth decade or beyond.4

For some young patients, however, tremor can be one of the most troublesome Parkinson's disease symptoms. Most Parkinson's disease patients experience an exacerbation of tremor and other Parkinson's disease symptoms with increased stress. This situation creates an escalating cycle of increased symptoms causing increased stress causing increased symptoms, which can lead to isolation and depression.

Other cardinal symptoms--such as bradykinesia (slowness of movement), gait difficulty, and rigidity--are seen in all age groups. Dystonic spasms (sustained abnormal postures, such as turning in or arching of the foot and toes) are more common in the young-onset patient and often precede the emergence of other, more typical features of the disease.5

As is the case of older-onset Parkinson's disease, the speed and severity of the progression of young-onset Parkinson's disease can vary greatly among individuals. Although the neuropathology and most clinical symptoms are the same at whatever age Parkinson's disease develops, the psychological, social, and medical management implications are very different in young-onset patients.

MEDICAL MANAGEMENT

The medical management of the young-onset Parkinson's disease patient requires an understanding of the significantly greater tendency of this group to develop dyskinesias or involuntary movements (most commonly dystonia) and motor fluctuations when taking levodopa. This medication is given most frequently for treating Parkinson's disease symptoms; therefore, it becomes mandatory to employ all levodopa-sparing strategies available when attempting to treat symptoms in the young Parkinson's disease patient. Such strategies include the use of anticholinergics or amantadine as well as substantial use of the dopamine receptor agonists for more potent, symptomatic treatment. Often these drugs are used in combination.

Nevertheless, levodopa is the most efficacious drug in all Parkinson's disease patients. It should be used at the lowest doses possible at the appropriate time--that is, when an inadequate response is obtained with the use of other medications or when side effects are encountered at doses necessary to achieve a desirable response.6

It is in the patient's best interest to seek a physician who has an understanding of the unique requirements of the young Parkinson's disease patient. Such an approach will allow long-term control of symptoms and maximization of functional independence.

PSYCHOLOGICAL AND SOCIAL ISSUES

It is difficult for the patient to receive the diagnosis of this chronic, neurodegenerative disorder at any age, but to grapple with its impact at a young age is hardly imaginable. The future may seem jeopardized by uncertainties: "What can I expect? Will I be able to continue working? What kind of medical bills can I expect? Will I still be able to function as a nurturing parent and spouse?"

The patient may experience a roller coaster of emotions as he or she and the family come to terms with the diagnosis. As the disease progresses, the necessity for physical and emotional adaptation presents daunting challenges and can take a toll on intrafamilial relationships. Special attention must be paid to the effects of Parkinson's disease on the family.

Employment

The most commonly asked question by the newly diagnosed patient is "How long will I be able to work?" For those who have struggled with the disease for a decade or more, the question may be "Should I keep working?"

Fortunately, levodopa therapy as well as other new Parkinson's disease medications have greatly improved treatment and delayed difficulties for the majority of patients. Newly diagnosed patients need to be reminded that although their body may not be acting in its usual, healthy way, Parkinson's disease progresses slowly. With currently available treatments, they will most likely have many work years ahead. In later years, the decision as to how long to continue to work or when to apply for medical disability is up to the individual.

Each person with Parkinson's disease presents a unique combination of symptoms, work-related issues, and family and financial circumstances. Decisions should be approached with the support of family, physician, and employer. In this way, the best choices can be made for each person.

Financial Planning

Financial counseling is helpful for the young person who may be anticipating a future with the increased financial demands of a growing family and the possible decrease in financial resources available to meet family needs. Insurance agents, financial planners, attorneys, and investment counselors can be helpful consultants in protecting assets and planning for the financial long term.

Children's Needs

Parkinson's disease is a family affair. Children will raise questions and concerns related to having a family member with Parkinson's disease: "Is it contagious? Is my parent going to die? Was it caused by something I did? Will it get better?"

The lack of facial expression exhibited by most Parkinson's patients makes children wonder if their parent is continually angry or sad. Children need to be reassured and to have their questions answered directly and honestly. Older children require concrete information about the disease as well as emotional support. Educational materials are available from the library or from any of the national Parkinson's disease organizations.

Depression

Estimates of the occurrence of depression in the Parkinson population run as high as 90%.7 Depression, which accounts for the majority of psychiatric referrals in patients with Parkinson's disease, can occur years before Parkinson's disease is diagnosed and may actually be the first indication of internal, neurochemical changes. It is thought that depression is overlooked in the Parkinson's disease population because many of the symptoms of depression listed below are also symptoms of the disease:

* Fatigue

* Slowness of movement

* Flat or masklike facial features

* Forgetfulness

* Difficulty concentrating

* Sleep disturbances

The patient should be evaluated carefully for depression, and antidepressant medication may be prescribed. Monoamine oxidase inhibitors and certain types of antidepressants should be considered carefully in patients taking selegiline hydrochloride.8 It is always advisable to check with a physician who is familiar with potential interactions of medications.

It is also important to be alert for symptoms of depression in the caregiver. The demands on the caregiver of adapting to shifting family dynamics and increasing physical, emotional, and financial responsibilities can cause depression and burnout. Both the patient and the caregiver may benefit from medication and counseling.

Sexual Dysfunction

Depression can markedly reduce libido and have a significant impact on sexual activity in people with Parkinson's disease. Sexual partners also may experience depression and fatigue as they struggle with the caregiving role in the relationship. Thus, they also may not have the energy for or interest in engaging in sexual activity. In addition, autonomic dysfunctions that result in drooling, excessive sweating, or excessive facial oiliness may interfere with the perceived attractiveness of the partner with Parkinson's disease.9

Health care providers should include questions regarding intimacy and sexuality when evaluating the patient's symptoms and quality of life. The issues surrounding sexuality in Parkinson's disease are complex and may involve both physiologic and psychological components. A referral to a urologist, gynecologist, or sex therapist can assist in identifying issues affecting sexuality.

Education and Support The issues described above are only some of the areas that challenge young people with Parkinson's disease. Education and support for all members of the family are essential.

In recognition of this need, the American Parkinson Disease Association (APDA) has established a Young-Onset Parkinson's Disease Information & Referral Center (see box), which addresses the unique issues concerning this population in a variety of ways. Under the direction of the Center's coordinator, who is a registered nurse and a licensed clinical social worker, toll-free telephone calls related to young-onset Parkinson's disease are answered from across the country.

Packets of educational and supportive materials--including The Young Parkinson's Handbook: A Guide for Patients and Their Families4--are sent free of charge to anyone requesting information.Edited by a young patient with Parkinson's disease who was diagnosed at age 38, and published by the American Parkinson Disease Association, this handbookaddresses the many everyday challenges young patients face personally, socially, economically, physically, and medically in dealing with the disease.

The Center also publishes the APDA Young Parkinson's Newsletter,6 which offers education, resources, and encouragement and targets the special concerns of the younger population. Recent newsletters have addressed such topics as parenting with Parkinson's disease, retirement planning, and impotency, and have included a column that provides an opportunity for children's voices to be heard.

In addition to written materials, the Center offers referrals to nationwide young-onset Parkinson's disease support groups, to physicians and other medical and mental health professionals who have experience with Parkinson's disease, and to a wealth of other resources. Support groups offer an excellent source of education, encouragement, resources, tips for adapting to Parkinson's disease, and the opportunity for socialization; however, some patients are reluctant to attend meetings.

For those who prefer one-on-one contact rather than or in addition to a group, the Center offers a program called "Person-to-Person Network," which provides participants with names of other young people with Parkinson's disease who have similar interests and who have agreed to be contacted.

CONCLUSION

Those affected by Parkinson's disease at younger ages comprise a special group. When attention and support are given to the many important issues that may arise over the years of living with this disease, long-term psychological and medical health can be maximized. n

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References

1. Parkinson J. An Essay on the Shaking Palsy. London, England: Sherwood, Neely & Jones; 1817.

2. World Health Organization. International Statistical Classification of Diseases and Related Health Problems. 10th rev. Geneva, Switzerland: WHO; 1992.

3. Duvoisin R. Parkinson's Disease: A Guide for Patients and Their Families. New York: Raven Press; 1991.

4. Koller W, Hubble J. Young-onset Parkinson's disease. In: Johnson A, ed. Young Parkinson's Handbook. Staten Island, NY: APDA Publications; 1995: 10-15.

5. Giovannini P, Piccolo I, Genitrini S, et al. Early-onset Parkinson's disease. Move Disord 1991;6(1):36-42.

6. Rezak M. Parkinson's disease in the young. APDA Young Parkinson's Newsletter Summer/Fall 1998:4.

7. Fitzsimmons B, Bunting L. Parkinson's disease: Quality of life issues.
J Neurosci Nurs 1993;28(4):807-817.

8. Rezak M. Ask the expert: Update on medical treatments for Parkinson's disease. Paper presented at: American Parkinson's Disease Symposium; March 14, 1998; Chicago, IL.

9. Brown R, Jahanshahi M, Quinn N, et al. Sexual function in patients with Parkinson's disease and their partners. J Neurol Neurosurg Psychiatry 1990;3:480-486.

About the Author:

Ms. Reese is Director of the Young-Onset Parkinson's Disease Information & Referral Center of the American Parkinson Disease Association. Address for correspondence: Susan Reese, RN, LCSW, MA, Glenbrook Hospital, 2100 Pfingsten Rd, Glenview, IL 60025. E-mail: APDA YPD@aol.com.