Rare Parkinson's Heroes Who Changed the World
Mr. Lloyd Tan is the icon and hero of the Parkinson's community. His spirit lives in us forever. Let us feel his energy as he guides us to live with Parkinson's, reaches out to us to give hope and inspiration.

Parkinson’s is generally known to be an illness of the elderly people. However, many people do not realize that even the young are affected by Parkinson’s.

In juvenile parkinsonism (JP), the symptoms start before the age of 21. Due to its strong genetic factor, JP usually affects members of the same family. JP is very rare in Western countries. I have never seen such a case in Malaysia. As such, JP is excluded from the present discussion.

The term “young-onset Parkinson’s” which is used in this article (and throughout the whole book) refers to the young-onset Parkinson’s (YOPD) patients, whose symptoms begin between the age of 21 and 40. About 5% – 10% of all Parkinson’s patients belong to the category of YOPD.

The term “older-onset Parkinson’s” refer to the more frequently seen Parkinson’s patients, whose symptoms start beyond the age of 40, and most commonly after the age of 60.

YOPD

I always remember my youngest ever Parkinson’s patient. He was just 23 when he started having the symptoms of Parkinson’s. He was studying an engineering course in the United States when he was struck by Parkinson’s. Even before he completed his study, he was forced to return to his hometown in Malaysia.

Four years ago, I received a phone call from his mother who said, “Please help my son.” I advised her to bring him to my clinic for treatment. But till today, he has not turned up. I guess he must have given up on his life. It must have been extremely hard for him to accept his illness at such a young age.

Over the recent years, I have met more and more of the YOPD patients. Compared with the older-onset Parkinson’s patients, I find that many YOPD patients have a stronger tendency to conceal their illness and avoid the general public (which is natural). This observation makes me wonder whether YOPD may be more common than what we observe. It must be painful to admit to the world that they have such an illness at a “wrong” age.

 

A unique group with special needs

In many aspects, the YOPD patients are similar to the older-onset Parkinson’s patients, in terms of symptoms and changes in the brain (Schrag A, 2006). However, due to some characteristic features of the YOPD patients, as described in the following pages, they are best regarded as a special subgroup of Parkinson’s patients which requires a different approach.

The YOPD patients need special attention

Medical aspect of YOPD

As in the older-onset Parkinson’s, the YOPD patients present with slowness of movement, tremor and body stiffness. However, foot dystonia as a presenting symptom is more common in the YOPD patients.

However, the YOPD patients tend to progress at a slower rate than the older-onset Parkinson’s. The more rapid progression of illness in the older-onset Parkinson’s has been attributed to the more extensive degenerative changes (process of brain cell loss) in the elderly people. As such, the YOPD patients live longer with the illness, up to 30 years.

The YOPD patients are also more likely to develop severe motor complications (especially dyskinesia) than the older-onset Parkinson’s patients. As the YOPD patients have a long duration of illness, the motor complications have a significant impact in their lives. In addition, the severe dyskinesia can cause embarrassment and contribute to the social stigma and isolation.

Lastly, there is a higher chance of inheritance among the YOPD patients compared with the older-onset Parkinson’s patients. Genetic testing can potentially identify family members at risk of developing YOPD. Nevertheless, genetic testing is not available in Malaysia, as in most other countries.

 

Young-onset Parkinson’s in Malaysia – it does not spare any ethnic group

Left: Ms. Khoo Siew Lay, Chinese, 50 years old, Parkinson’s since age 38.
Centre: Mdm. Zanariah Ali, Malay, 33 years old, Parkinson’s since age 26.
Right: Mr. Pandirajan, Indian, 43 years old, Parkinson’s since age 32.

 

The psychosocial aspect of YOPD

Compared with the older-onset Parkinson’s patients, the YOPD patients face more severe psychosocial problems. To begin with, it is already so hard for the YOPD patients to accept an “elderly people’s illness”. As the YOPD patients start having the illness since a young age, they have to endure many more years of suffering than the older-onset Parkinson’s patients.

Moreover, the YOPD patients are still in the “prime time” of their lives, being married with young children and economically productive. The high level of expectation from family members, especially in meeting the financial needs of schooling children, can be a source of significant stress to the YOPD patients.

It has been reported that the YOPD patients are more likely to be unemployed or retire early from their jobs, unable to drive and experience more marital and family problems (Schrag A, 2006). In addition, the YOPD patients perceive more stigmatization, are more depressed and have poorer quality of life than the older-onset Parkinson’s with same illness severity.

The lack of emotional support is another important contributing factor to psychosocial problems of the YOPD patients. Almost all of the YOPD patients whom I know are reluctant to join the Parkinson’s support group as they know that they will be made to feel “out of place and time”, while facing the majority of Parkinson’s patients who are in their late ages. The meeting with the “senior citizen group” of Parkinson’s patients further remind them of how unfortunate they are in having an “elderly people’s illness” when they are still young. Furthermore, the very sight of physically immobile and severely trembling patients in the advanced stage of illness, especially when they are on the wheelchair, is very discouraging for these YOPD patients.

Sex life

Being in the age group where people are most sexually active, any difficulty in maintaining a successful sexual relationship has significant impact on the YOPD patients and their marital relationship. The impairment of body movement (especially fine hand movement) due to tremor and stiffness, severe dyskinesia and erectile dysfunction (commonly due to depression), can do a lot of damage to their self-esteem.

I can always vividly recall a YOPD patient who became severely depressed when he was diagnosed to have Parkinson’s. A few months before he met me, he had already consulted another Neurologist, who advised him to start taking medications. But he refused to do so, as he was still in a state of denial.

I spent about one hour listening to his grievances in my clinic. He told me about how his life had been turned upside-down due to the illness. He was particularly worried about the future of his career – he was a very outgoing man, who took part actively in driving expeditions to the jungles.

 

Another issue which really bothered him was his sex life. He said, “My self-esteem is badly affected. In the sexual relationship with my wife, I have always been the leading person. I believe that as men, we are the ones who usually initiate the foreplay and dominate the whole love-making process. But now, the stiffness and clumsiness of my hands make it so difficult to caress my wife. The very sight of my trembling hands itself is depressing enough. I have this crazy idea that if I continue to “under-perform”, my young wife will look for another new partner.”

Just as the first doctor whom he had consulted earlier, I advised him to start taking medications. But that was the first and last time that I met him. He must have remained in a state of denial.

 

Pregnancy

Pregnancy is a highly relevant issue in the YOPD patients. Overall, pregnancy is rare in Parkinson’s, probably because some young patients may decide not to get married or pregnant.

In my entire career, I have met only one Parkinson’s patient who had successfully given birth when she was 28, two years after she was diagnosed to have Parkinson’s. She is Mdm. Zanariah Ali, a 33 year-old lady with YOPD (please refer to the article “Parkinson’s patients have more than one life” later in this chapter). Two weeks after she missed her period, she informed her doctor about her pregnancy. She was advised to immediately stop all her Parkinson’s medications, right up to the day of delivery. Fortunately, the delivery was uneventful – she did not need any specific procedure. Her five year-old son is perfectly healthy.

In general, it is still not known whether pregnancy has any definite negative effect on the symptoms of Parkinson’s. In some patients, pregnancy does not aggravate the symptoms of Parkinson’s. However, it has been reported that Parkinson’s can be temporarily or permanently worsened during pregnancy (Schulman LM, 2000).

Due to the small number of reports of pregnancy in Parkinson’s, it is difficult to confirm the safety of Parkinson’s medications during pregnancy. In general, regardless of whether someone is healthy or suffers from Parkinson’s, doctors would advise patients to avoid any medications during pregnancy. In reality, it is quite difficult to ask a patient to discontinue the Parkinson’s medications as it will worsen the symptoms of Parkinson’s. As such, Parkinson’s patients who plan to be pregnant are advised to consult the relevant specialists before making the final decision.

The important aspects of the daily life of the YOPD patients such as occupation, driving, sex life and social life are further discussed in the following articles.

YOPD needs an “all-out” treatment

The YOPD patients need strong support in every aspect of their life, especially the medical and psychosocial issues. Obviously, a multi-disciplinary approach is needed for optimal management of YOPD patients. This “YOPD Team” should include occupational therapists, physiotherapists, Rehabilitation Medicine specialist, social workers, psychiatrists, Neurologists and Neurosurgeons.

In the medical treatment of the YOPD patients, it is important to delay the use of levodopa as long as possible, due to the high risk of developing motor complications. Treatment should start with a dopamine agonist, in order to postpone the onset of motor complications.

Generally, the treatment of YOPD should be “aggressive”, so that they can maintain their physical, mental, occupational and social function as long as possible. The word “aggressive” refers to the use of a relatively high dose of medications (in combination) and a lower threshold for recommending the DBS surgery.

Compared with the older-onset Parkinson’s, it is advisable that the DBS surgery for YOPD is performed at an earlier stage of illness (please refer to the article “Back to the future” in Chapter 15). Even if their symptoms are just moderately severe, YOPD patients should be considered for the DBS surgery when their daily activities, especially their career, are significantly impaired. In these cases, the DBS surgery offers not only symptomatic relief, but also a better ability to maintain their career and all other daily activities, at the most optimal level of functioning. In addition, the relatively early DBS surgery may prevent the psychosocial complications associated with YOPD.

Summary

  • Parkinson’s also affect the young people: about 5% – 10% of Parkinson’s patients belong to the YOPD category (onset of symptoms between age 21 and 40)
  • Compared with the older-onset Parkinson’s, the psychosocial impact on YOPD is greater, resulting in higher rates of unemployment, marital problems and depression
  • The YOPD patients are a unique subset of Parkinson’s which needs special attention and a more “aggressive” treatment, so that they can maintain their physical, mental, occupational and social function in the most optimal manner
  • For the YOPD patients, the DBS surgery should be performed at an earlier stage of illness in order to prevent or minimize the sychosocial complications

References

Schrag A, Schot JM. The epidemiological, clinical and genetic characteristics of early-onset parkinsonism. Lancet Neurol 2006; 5: 355-63.

Schulman LM, Minagar A, Weiner WJ. The effect of pregnancy in Parkinson’s disease. Mov Disord 2000; 15: 132-135.