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1st Oct 2009.

I had “Parkinson’s” since I was 16

One month ago, a lady came to my clinic and said, “I was only 16 when I developed slowness of movement, leg tremor and body stiffness. A few years later, the neurologist told me that I had “Parkinson’s”. My 25-year-old brother also had “Parkinson’s” since he was 18.”

If you had heard this from this lady, you must have thought she had got the numbers (i.e. ages) wrong.

In view of the general perception that Parkinson’s is an illness that “belongs” to the elderly people, it is usually hard to believe that young people are also affected by the same illness, especially teenagers.

That unusually young “Parkinson’s patient” was Ms. Goh Mei Ling, a 36-year-old lady whom I had met the first time recently.

You have heard of YOPD patients (symptoms of Parkinson’s start between the ages of 20 to 40), the most remarkable of whom is Mr. Michael J Fox. But the “Parkinson’s” that Ms. Goh is suffering from is actually a brain disorder which is termed Juvenile parkinsonism (JP), in which the symptoms such as slowness of movement, body stiffness and tremor start before the age of 20 (JP also occurs in children!). She is the first and only patient with JP whom I have met in this country.

JP is commonly caused by genetic factor (i.e. there is a strong role of inheritance). As such, frequently there is another (or more) family member who is affected by the same illness. The percentage of familial occurrence in JP is higher than in YOPD. The major genetic abnormality in JP has been shown to be parkin gene mutation (Lucking CB, 2000). In these cases of JP due to parkin gene mutation, the response to levodopa is generally good while dyskinesia tend to occur at early stage of illness.

JP is generally rare, and seems to be more common in Japan. The higher frequency of JP in Japan may be related to higher percentage of consanguinity in the Japanese population (Schrag A, 2006). In Ms. Goh’s case, she clarified that her parents are not genetically related (not cousins). She has a total of six siblings. Apart from Ms. Goh and her younger brother, there is nobody else in the family who is affected by the brain disorder.

Even though her symptoms started at the age of 16, she was diagnosed to have ‘Parkinson’s” only a few years later. “During the first three years of my illness, I consulted many doctors at the local hospital. Despite this, none of them told me I had “Parkinson’s”. They gave me some medications which did not help at all,” recalled Ms. Goh. I was not surprised to hear this because there is commonly a delay in the diagnosis of YOPD and JP as many doctors themselves do not realize that even the young are affected by parkinsonism.

Subsequently, a neurologist in Kuala Lumpur told her that she had “Parkinson’s” and treated her with levodopa (Sinemet). “The response to medication was very good. I just needed half tablet of Sinemet (125) three times a day to make me feel almost like normal again,” said Ms. Goh.

Nevertheless, she had abandoned her studies at this stage. She was married at the age of 20. She was very fortunate to have a loving husband who accepted her illness, despite knowing that it was an incurable brain disorder. “I remembered that during our wedding day, my wife had severe cramp of the neck muscles that caused her to look upwards. She had to struggle to look at the camera when our wedding photograph was taken. You can see the bulging of her neck muscles which was caused by the muscle contraction,” recalled her husband as he pointed to the appearance of her neck in the wedding photograph.

The wedding day – Ms. Goh trying her best to smile on her wedding day. Notice the bulging of her neck muscles which was caused by the muscle contraction due to JP. Ms. Goh and her four-month old son.

 

By age 22, when she had been pregnant for five months, she consulted the doctors at the local hospital. The doctors warned her about the possibility of side effects of the Parkinson’s medications during pregnancy (i.e. birth defects) and risk of her child inheriting the same illness. Again, she had luck on her side. The delivery of her child was uneventful & she did not require any form of assistance or surgery. Her son, who is currently 14, is as healthy as other teenagers.

Ms. Goh with her husband (left) and her 14-year-old son (right).

Four years after the birth of her son, she became pregnant again. This time, both Ms. Goh and her husband decided that they would not want to take the risk again. The second baby was aborted.

During the first ten years of her illness, she could still cope with her daily activities. She could cook and perform household work during her “on” periods.

As expected, over the past five years, her disease has progressed significantly. She had frequent falls due to off-period freezing episodes. As such, she was not able to leave home on her own for five years. Even at home, she had difficulty carrying out daily activities. Her “on” period is merely two hours, during which she is also not very well. She needed to take one tablet of Sinemet (125) when she woke up, followed by half tablet of Sinemet (125 mg) once every two hours.

When I met her last week, I assessed her during the “off” and “off” period. When she was “off”, her body was very stiff and weak to the extent that she struggled to get out from bed. When she tried to walk, she was very slow and developed freezing. After taking 1 ½ tab of Sinemet (125), she was “on” and could walk around quite well without any freezing. In view of her good response to levodopa, I told her that she would benefit from the Deep Brain Stimulation (DBS) surgery.

I explained to her that the DBS surgery would help her to travel about 10 years back in time, i.e. after the DBS surgery, her illness would be as though it was ten years ago. Her husband agreed, and said, “she has been suffering a lot for the past ten years. If the surgery can help her to live almost a normal life again, why not go for it?”

She has been scheduled for the DBS surgery in March 2010. In the meantime, I have started her on some new medications, in order to optimize her condition. If the DBS surgery is successfully carried out next year, she will be the youngest patient in this country to have undergone this procedure.

Ms. Goh with her younger brother who also suffers from JP (left) and mother (right).

References

Lucking CB, Durr A, Bonifati V, et al. Association between early onset Parkinson’s disease and mutations in the parkin gene: French Parkinson’s Disease Genetics Study Group. N Engl J Med 2000; 342: 1560067.
Schrag A, Schott JM. Epidemiological, clinical, and genetic characteristics of early-onset parkinsonism. Lancet Neurol 2006; 5: 355063.