Understanding Parkinson's — The shaking palsy

MsT sheds light on Parkinson’s disease — one of the most disabling of physical conditions


Creative: Omer Asim/Ans Khurram January 19, 2015
DESIGN BY OMER ASIM

Back in the year 1817, English surgeon James Parkinson published a research paper titled An Essay on the Shaking Palsy. Herein, he systematically described a condition which caused characteristic paralysis, diminished muscle strength, abnormal postures and resting tremors in six individuals he had studied.

Not only did James manage to distinguish this special type of tremors from other bodily tremors, he also found that the symptoms exhibited by his subjects progressed over time. James referred to the condition that would later bear his name as paralysis agitans or the shaking palsy. Although he died seven years after, his research was continued and 60 years later, the condition was given the name of Parkinson’s disease.

Now, nearly 200 years after James’s discovery, Parkinson’s has become the second most common neurodegenerative disorder in the world, affecting approximately seven million people globally. Out of this, one million patients hail from the United States alone. According to an estimate issued by the Pakistan Parkinson’s Society, about 400,000 people suffer from Parkinson’s in Pakistan. Unfortunately, a large part of our society remains unaware of the diseases completely, let alone understands its causes, symptoms and treatments.

What is Parkinson’s disease?

In a nutshell, Parkinson’s is a degenerative disorder of the central nervous system.  It is the most common form of ‘Parkinsonism,’ an umbrella term for a group of motor system disorders sharing four primary symptoms: tremors, slowness in movement, postural instability and rigid limbs. According to Dr Zafar Iqbal, professor of neurosurgery and founding member of the Zafar Brain and Spine Clinic in Lahore, “Parkinson’s is both chronic and progressive in that is persists over a long period of time and escalates as time goes by. Fortunately, though, it is neither contagious nor hereditary.”

Upon onset, the most discernible symptoms of Parkinson’s are usually movement related but as it progresses, a patient may experience thinking and behavioural problems like depression and even dementia. “It took me over a year to realise something was wrong as I couldn’t exert the same amount of force with my hands,” shares 51-year-old architect Humeira Naim. “My movement was compromised and I could barely hold things by the time I was finally diagnosed in 2010.” Sixty two-year-old accountant Jamal Khalid had a similar experience in that he sensed something suspicious while driving. “I started having trouble changing gears,” he recalls. “Also, my writing had become slanted and illegible. That was when I realised I might have Parkinson’s.”

What causes Parkinson’s disease?

Initially, James predicted that the shaky movement and tremors experienced by the patients were caused by lesions in the cervical spinal cord. He was, however, proven wrong in the early 60’s by researchers that indentified a fundamental brain defect that is the hallmark of Parkinson’s disease: the loss of dopamine which promotes muscles activity.

“Dopamine is a chemical messenger of sorts which transmits signals from the brain to the rest of the body to induce smooth and meaningful muscle activity,” explains Dr Zafar. “The loss of dopamine causes the nerve cells in the brain to lose control, thereby making it difficult for patients to control their movements.” Typically, patients suffering from the disease have lost 80% or more of their dopamine-producing brain cells. Unfortunately, the cause of cell impairment or cell death still remains unknown, rendering Parkinson’s an idiopathic disease.

Do gender and age matter?

According to studies like the Clinical Spectrum of Parkinson’s Disease from Pakistan, 63% of the patients diagnosed are male. Another research released by the UK Parkinson’s Disease Society Brain Bank, showed that age plays a key role in diagnosis and treatment as the disease generally occurs in people over the age of 50. The average age upon diagnosis is 60 years but there have been a few, sporadic cases of patients exhibiting symptoms before the age of 40.

Nonetheless, Parkinson’s affects everyone differently and can progress much faster in some patients than others. For Humeira, walking has now become a luxury. “Parkinson’s has affected my ability to use my legs the most,” she says. “Not to mention, my right side — hands and leg — has deteriorated more than the left.” In Jamal’s case, Parkinson’s has impaired his speech and most recently, sparked skin rashes. “My voice has become slightly raspier and high in pitch, I think,” he claims.

Could you have Parkinson’s disease?

A range of symptoms follow, some of which are minor and ignorable while the others can be rather bothersome. Of course, it is difficult to predict which symptoms will manifest themselves in an individual patient or how intense they might be but most of them can be treated with appropriate medication or physical therapy. According to the Pakistan Parkinson’s Society, the motor symptoms (movement related) include

•  Tremors: These can be rhythmic, back-and-forth movements of the thumbs and forefingers, also called ‘pin rolling.’  Tremors are rarely disabling and usually disappear during sleep or improve with intentional movements.

•  Rigidity: In normal human beings, movement occurs as a result of one muscle becoming active and its opposing muscle relaxing. In Parkinson’s disease, however, this balance is skewed, causing the muscles to become tense and contracted perpetually. As a result, the limbs become weak and patients experience chronic aches.

•  Bradykinesia: This refers to the slowing down and loss of spontaneous and automatic movement in the human body. Bradykinesia is quite unpredictable and therefore, extremely problematic in that routine activities (such as washing or dressing) that were once done quickly can now take up to several hours to complete.

•  Postural instability: Typically a feature of late-stage Parkinson’s, postural instability hinders structural balance and causes frequent falls and bone fractures. Many patients also develop stooping wherein the head is bowed and shoulders drooped, with the abdomen bent forward all the time.

•  Retropulsion and festination: These affect the walking movement of patients directly. Retropulsion causes a backward lean in the body as patients tend to step backwards when walking. Festination, on the other hand, makes patients walk in a series of small, quick steps as if hurrying forward in order to maintain balance.

Can Parkinson’s be managed or treated?

Dr Zafar says that although there is yet to be a breakthrough cure for Parkinson’s, most of the symptoms can be curtailed using different medicines, surgery and multidisciplinary management. Both Humeira and Jamal, for instance, get a variety of treatments to ease their condition. And when medication does not suffice, surgery and deep brain stimulation (DBS) can be helpful.

Levodopa (also called L-Dopa) is commonly administered in order to replenish the amount of Dopamine in the brain. Unfortunately, only about 10% of the drug crosses the blood-brain barrier as most of it is metabolised to Dopamine elsewhere in the body. This can perpetrate nausea and stiffness in the bones. Dr Zafar suggests Levodopa should be combined with another drug called Carbidopa which, he says, “Delays conversion until the Levodopa has reached the brain cells.”



Of course, even a strong combination of drugs can prove futile in many patients like Humeira who claims homeopathic medicine helped her more. “I take Levodopa and Carbidopa with Dopamine agonists but they didn’t help me as much,” she explains. “Homeopathy, however, helped me regain circular movement in my arms and has fewer side effects too.” For Jamal, the same medication actually sparked dyskinesia (involuntary muscle activity) and he began to have involuntary movements in his left foot.

During the initial stages of Parkinson’s, all treatment should aim to achieve the optimal balance between managing the symptoms and side effects. The administration of Levodopa may be delayed via other medications like MAO-B inhibitors and Dopamine agonists. Surgery is sought only if the drugs given are no longer sufficient and less than 10% of all Parkinson’s patients qualify for it. “Initially, we would perform a thalamic surgery on the brain but that is dangerous,” says Dr Zafar. “Now, we usually opt for deep brain stimulation wherein we drill a hole in the skull and insert an electrode that transmits electric impulses to certain parts of the brain.” Other than DBS, ablative surgery (the irreversible burning or freezing of brain tissue) and transplantation/restorative surgery are two more options.

Nonetheless, Parkinson’s disease calls more for palliative care to improve quality of life than anything else. Exercise and physical therapy, for instance, has proved to improve motor function in many patients like Jamal, even in the final stages of the disease. “I walk on a treadmill for at least an hour, every day and it has helped reduce the rigidity in my body,” he says. Humeira also indulges in yoga daily to maintain her flexibility.

Sadly, Pakistan needs to improve its current palliative facilities to cater to the growing demand. “There is a lack of neuro-physicians here,” says Dr Zafar. Only a 100 neuro doctors cater to a population of 180 million! Fortunately, the drugs for Parkinson’s are relatively inexpensive and hospitals across the country are striving to become better. For instance, the Lahore General Hospital recently launched DBS but the treatment remains too expensive for most patients.



Inspiration for those suffering from Parkinson’s

Micheal J Fox (Actor)

Many celebrities across the world suffer from Parkinson’s and prove that you can lead a healthy life, even after diagnosis. One such example is veteran Hollywood actor Micheal J Fox who was diagnosed in 1991 and semi-retired from acting as his disease worsened. He has since become an advocate for research to find a cure for Parkinson’s. In fact, Micheal has even established the Micheal J Fox Foundation and was awarded the honoris causa degree by Sweden’s Karolinska Institutet for his efforts in March, 2010.

Inspiration for those suffering from Parkinson’s

Mohammed Ali (Boxer)

Mohammed Ali, who is considered to be one of the greatest boxers of all time, suffers from Parkinson’s and claims it to be his “toughest fight.” He has also established the Mohammed Ali Parkinson Centre in Pheonix, Arizona which serves as an outstanding resource for patients suffering from movement disorders. Both Michael and Mohammed Ali have gone on record to say that realising Parkinson’s can’t be cured and only managed is important for patients to develop a positive outlook and lead productive lives.

Non-motor symptoms of Parkinson’s disease

There are some neuro-psychotic disorders and bodily impairments that can serve as warning signs for early Parkinson’s. These include:

•  Depression

•  Memory loss

•  Slow thinking

•  Intellectual loss and dementia

•  Changes in speech

•  Difficulty in chewing and swallowing

•  Urinary and digestive problems

•  Disrupted sleep patterns

•  Excessive sweating

•  Skin problems

Published in The Express Tribune, Ms T, January 18th, 2015.

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