A Parkinson Disease Primer

 

What Is Parkinson Disease?

Parkinson disease was discovered by the English physician, Dr. James Parkinson, in 1817.  Parkinson first wrote of this occurrence as “Shaking Palsy.”  Parkinson disease (PD) is a chronic, progressive, neurodegenerative disease that affects the substantia nigra portion of the brain.   The four major symptoms characterizing Parkinson disease are:

  •  Tremor
  • Muscle rigidity
  • Bradykinesia (slow movement),
  • and Postural imbalance

When at least two of these symptoms are present, and especially if they are more evident on one side than the other, a diagnosis of PD is made, unless there are atypical features that suggest an alternative diagnosis. Patients may first realize something is wrong when they develop a tremor in a limb; movements are slowed and activities take longer to perform; or they experience stiffness and have balance problems. Initially, symptoms are a variable combination of tremor, bradykinesia, rigidity, and postural instability.  Symptoms typically begin on one side of the body and spread over time to the other side.


Other signs of Parkinson disease may include:

  • Small, cramped handwriting
  • Stiff facial expression
  • Shuffling walk
  • Muffled speech
  • Depression

Changes occur in facial expression, so that there is a certain facial fixity (blank expression showing little emotion) or a staring appearance (due to reduced frequency of eye blinking). Complaints of a frozen shoulder or foot drag on the affected side are not uncommon. As symptoms come on gradually, older patients may attribute these changes to aging. The tremor is thought to be “shakiness,” bradykinesia is regarded as normal “slowing down,” and stiffness is attributed to arthritis. The stooped posture, common to PD, may be attributed to age or osteoporosis. Both younger and older patients may experience initial symptoms for a year or more before seeking medical evaluation.


Who does it affect?

Parkinson disease affects about 1 in 300 people in the general population. The occurrence increases with age to 1 in 100 at the age of 65 and to about 3 in 100 at the age of 85. There are currently between 500,000 and a million people in the United States with Parkinson disease. Most people are diagnosed with the disease between the ages of 50 and 60 and about 85 percent of people with Parkinson are over the age of 65. There are approximately 60,000 new cases of PD diagnosed each year and this number is increasing yearly. Men are almost 1.3 times more likely to be diagnosed than women.

 

What causes PD?

The cause for Parkinson disease has not been established.  A primary risk factor for PD is age. There also appears to be a genetic predisposition that when combined with an environmental factor causes extreme neuron death in the part of the brain that controls movement. Some of the environmental toxins include:

  • Certain industrial concentrated pesticides and fungicides
  • Repeated exposure to some heavy metals
  • Viral Infections
  •   Increased oxidative stress

 

When the cause of Parkinson disease is not associated with any of these factors, it is termed idiopathic (meaning “unknown cause”) Parkinson disease.  This category makes up about 85 percent of those with Parkinson disease.  

 

How do you get it?

The cells in the part of the brain called the substantia nigra produce a neurotransmitter called dopamine. Dopamine is necessary for motor neurons to communicate with each other to initiate and continue ordinary movements. As people age, some of the cells die off naturally. In most people, approximately 35 percent of these dopamine-producing cells are gone by age 60.  By the age of 80 to 90, over three quarters of these cells have died, leaving a diminished number of cells to supply other parts of the brain with dopamine. In people with Parkinson disease, the cells die much more prematurely than usual, creating dopamine deficiency at an earlier age.

 

This profound shortage of dopamine leaves brain cells unable to pass along the message of what to do in areas of the brain that normally regulate such vital skills as standing, sitting, and walking.

 

When a person develops symptoms of PD, it means that there has already been severe loss of cells in the substantia nigra, probably around 75 to 80 percent. Depending on which nerves are lost, symptoms may start in different parts of the body on each side.

 

How does it progress?

Initially the symptoms are mild, usually on one side of the body, and may not require medical treatment.  Resting tremor is a major characteristic of PD, and the most common presenting symptom, but some patients never develop it.  Tremor may be the least disabling symptom, but is often the most embarrassing to the patient.  Patients may keep their affected hand in their pocket, behind their back, or hold something to control the tremor, which may be more psychologically distressing than any physical limitation that it imposes.

 

Over time, initial symptoms become worse. A mild tremor becomes more bothersome and more noticeable. Difficulties may develop with cutting food or handling utensils with the affected limb. Bradykinesia (slowness in movement) becomes a significant problem and the most disabling symptom. Slowness may interfere with daily routines; getting dressed, shaving or showering may take much of the day. Mobility is impaired and difficulty develops in getting into or out of a chair or a car, or turning over in bed. Walking is slower and there is a stooped posture, with the head and shoulders hanging forward. The voice becomes soft and monotone. A disturbance of balance may lead to falls. Handwriting becomes small (“micrographia”) and illegible. Automatic movements, such as arm swing when walking, are reduced.


Symptoms may originally be restricted to one limb, but will typically spread over time to the other limb on the same side. They eventually progress to the other side of the body.  Generally this progression is gradual, but the rate of progression varies in different patients. 

It is not possible to predict with any confidence the likely course of the disease in an individual patient. The rate of progression and resulting level of disability vary from patient to patient. Some indication of the likely outcome in individual patients is provided by its progression since first being diagnosed, but this is only suggestive of te future course.

Is there a cure?
To date there is no cure for Parkinson disease. In addition, because there is no definitive cause for the disease, it is not preventable. The management of Parkinson disease is based on relieving the symptoms as much as possible. The management of the disease can include: harmaceutical therapy, vitamin supplementation, good nutrition, exercise, and in some cases,surgery.

There are a number of effective medicines that ease the symptoms of Parkinson disease.  Most symptoms are caused by lack of dopamine.
The medicines most commonly used will attempt to either replace or mimic dopamine, which improves the tremor, rigidity, and slowness associated with Parkinson disease.  Several new medicines are being studied that may slow the progression. Many promise to improve the lives of people with Parkinson disease. 
A relatively new surgical procedure that was approved by the FDA in 2002 can reduce symptoms and reliance on medications.  This procedure called “Deep Brain Stimulation” inserts two small electrodes into a portion of the brain that regulates much of the movement affected by PD.  The electrodes are connected to two pacemaker-type devices implanted under the skin in the chest that send small electrical impulses that may help relieve tremors, rigidity and slowness. DBS surgery can ease the symptoms of Parkinson disease, but it is not a cure. 

 

What’s in the future?

There is much research being done that scientists hope will lead to identifying the cause and, eventually, discovering a cure. Cell transplantation, gene therapy, and the injection of nerve growth factors may offer a potential cure in the future.