Lecture # 413:
Parkinson's Disease and Amyotropic Lateral Sclerosis

copyright 2008 Cheryl K. Hosken, BSN, MS Psych.

In 1817, James Parkinson, a British doctor described the symptoms of Parkinson's disease. He called it the "shaking palsy". It was not until 1950 that a Swedish scientist, Arvid Carlsson, discovered that people with Parkinson's disease are deficient in dopamine in the region of the brain called the straitum. Two Viennese neuroscientists found that giving levodopa improved the symptoms of these patients.

Cause of Parkinson's Disease
In the early 1900s, pathologists in Europe and the United States recognized a specific abnormality in the brains of individuals who in life had Parkinson's disease. The disease obliterates nerve cells in a broad area of the inner brain: the basal ganglia. It particularly destroys the dark pigmented neurons in a tiny, but essential segment called the substantia nigra. The nerve cells of the basal ganglia and substantia nigra produce the chemical dopamine. The dopamine helps the substantia nigra to interact with the striatum that is responsible for controlling movement, walking, and balance. There is an abundant reservoir of dopamine in this region, but when the level drops below 80%, symptoms of Parkinson's disease begin to emerge.

Meditate Word By Word On These Verses:
Eccles. 12:1-7.

The loss of dopamine causes the nerve cells of the basal ganglia to fire out of control, leaving patients unable to direct or control their movements in a normal manner. Why the neurons of the substantia nigra die in certain individuals and not others remains a mystery; however, recent advances in Parkinson's disease research suggest that environmental influences and an individual's genetic makeup may both play a role.

Being Diagnosed with Early Symptoms
Many people with Parkinson's disease go undiagnosed for a while because the typical complaints and findings are very subtle. The classic trio of Parkinson's includes tremors, muscular rigidity, and bradykinesia. Prior to the diagnosis of Parkinson's disease, a person may begin to feel a drop in energy or a loss of coordination. One of the first symptoms is impaired handwriting. After the first few words, the letters appear cramped. The second symptom is rigidity. There is a dynamic tension between muscles that generates movement. For example, the biceps contract while the triceps relax. But in Parkinson's the triceps do not relax and produce a stiff ratchet-like motion.

Bradykinesia or slow movement is the third symptom. This is a shuffling type of walk. Many times, Parkinson's patients experience bradykinesia of the nerves that control the facial muscles. This gives their faces a mask-like appearance. The person may have feelings, but is unable to show them. The condition may later affect the nerves and muscles necessary for speech. Then the voice has a flat, monotone sound. These symptoms evolve gradually and may even be imperceptible to the patient or family members until a physically or emotionally stressful event occurs, triggering an increase of these symptoms.

Disease Progression
Progression of Parkinson's disease is highly variable, although progression may be relatively slower in patients whose initial symptoms include tremor. When the disease is fully expressed, the major clinical features include:

  1. bradykinesia (slow movement),
  2. tremor (typically at rest and extinguished with movement) and,
  3. impaired postural reflexes (poor balance).

A variety of symptoms can be associated with Parkinson's disease:

Question 1: What are the three major features of Parkinson's disease?

 

 

Diagnosis
Most of the tests used to diagnose Parkinson's do so indirectly by ruling out other conditions that have identical characteristics. The most valuable way to diagnose the disease is by listening to the patient and examining him.

Treatment
The medication for treatment of Parkinson's disease is called dopa, L-dopa, or levodopa. This is a precursor of dopamine. The dopamine found in bananas and other fruits cannot get into the brain. The most widely used drug is called Sinemet. It contains a compound that prevents the kidneys and liver from breaking up the compound of dopa before it gets to the brain. This drug can cause hallucinations, vivid dreams, or nightmares. After about 3-5 years of treatment, the brain responds less and less to the drug. Then the person begins to have complications that hamper his mobility.

The complicated movements are:

Other forms of Parkinsonism * Postencephalitic Parkinsonism: Caused by a virus that resulted in a condition called encephalitis lethargica or "the sleeping sickness". Victims of this condition who recovered were often found to develop Parkinsonian features weeks to even years following recovery from the encephalitis. Neurologist Oliver Sacks in his book The Awakenings popularized this condition.

* Drug-induced Parkinsonism. A reversible form of Parkinsonism usually caused by certain drugs known as neuroleptics:

* Vascular Parkinsonism. Caused by an accumulation of small strokes. Anti-Parkinsonian medication offers little benefit. * Toxin-induced Parkinsonism:

Question 2: What other substances cause Parkinson's-like symptoms? What should you do if someone is displaying these symptoms?

 

 

TERMS USED TO DESCRIBE MOVEMENT DISORDERS
Akinesia: the inability to move.
Bradykinesia: literally slowed movement.
Dystonia: Involuntary contraction of a muscle or a group of muscles.
Dyskinesias: Abnormal involuntary movements that can be characterized as writhing movements and can include dystonic movements. These movements can be seen in a variety of disorders such as Huntington's Chorea, the dystonias and Tourette syndrome. These movements are commonly caused by levodopa and other antiparkinsonian medication and are often seen as a delayed reaction to antipsychotic medication such as Haldol.
Parkinsonism: A generic term referring to clinical findings similar to Parkinson's disease, but occurring in variety of other neurological disorders.
Ataxia: Ataxia is a disorder characterized by a lack of coordination, involving fingers, limbs, body, eyes, or speech. The condition can be inherited or a result of another medical condition, such as stroke, head trauma, or exposure to certain drugs or toxins.

Exercise for Parkinson's Disease Daily exercise can help reduce the disabling effects of Parkinson's. Exercise also enhances the effect of the medications the person takes. Since Parkinson's reduces the range of joint motion, the goal of exercise should be to extend, rotate, and flex the joints and muscles. For example, patients can improve their posture simply by reaching up the sides of a doorframe and gripping it.

There is a good twisting exercise that helps reduce stiffness during walking and can be used by persons who have had strokes as well. It also helps a person to roll over in bed. Here are the directions:

  1. The person sits in a chair with feet flat on the floor and arms folded across the chest.
  2. Ask him to turn his upper body to the left and bend forward until the right elbow touches the left knee.
  3. Return to an upright position.
  4. Repeat with the other arm to the opposite knee.
  5. Repeat 5 times.

To ease tremors, a person can lie face down on a bed and take deep breaths. The shaking will subside in 5-15 minutes. When hand tremors come on, give the person a rubber ball to squeeze. When he eats, show him how to grip the spoon firmly with the thumb, then curl the other fingers around the handle. Give him a half-full cup of liquid rather than full. A mug is better to drink from than a cup or glass. If the person is bothered by tremors at night, he should sleep on the same side as the tremor and put his hand under a pillow. The weight of the pillow should stop the tremor.

Because of the disease process, often persons with Parkinson's disease become inactive and stay at home. They need to be encouraged to walk as much as they can outside.

Question 3: What is helpful to Parkinson's patients for mobility?
(Only one of the following answers is correct.)
a wheelchair.
daily exercise.
a cold shower every morning.

 

 

Diet The protein in foods can interfere with the bloodstreams' absorption of levodopa. Some people find that if they take their medication after eating a meal with protein, the effect of the medication is stopped. If a person takes his levodopa in the morning, he should eat protein in the evening. Then throughout the day, his muscle activity will be smoother.

Due to the effects of Parkinson's, the stomach and digestive tract work more slowly. The food needs to leave the stomach to the intestines where the nutrients are absorbed and put into the blood for circulation. Therefore, the levodopa medication should be taken when the stomach is empty, at least 30 minutes before eating. To aid absorption of the medication, a person needs to move around after taking it. If the levodopa is crushed and taken in water, the absorption in the stomach is faster. There are also drugs that help the stomach muscles move better. One of them is Cisapride or Propulsid.

Long-Term Effects of Parkinson's Disease

Impaired Ability to Move
Often the tremors in the hands may remain about the same, but the ability to walk worsens. The person shuffles forward instead of walking. These people look like they are walking into a fierce wind - they use small steps and lean forward because their sense of balance is worsening. Their arms hang at their sides or are partially flexed. As you can imagine, these people are vulnerable to falls.

To prevent falls there are a few things you may do to help this person. Parkinson's patients tend to walk on the balls of their feet instead of the heels. You can do the following to help him stop this gait:

  1. Stand still.
  2. Spread you feet 8 inches apart.
  3. Think about taking a broad (wide) step.
  4. Lift one foot high, as if you are marching.
  5. Lift up the toes and set the heel down first.
  6. Roll onto the ball of the foot and toes.
  7. Do the same with the other foot.
  8. As you walk, swing arms in cadence - left arm with right foot, right arm with left foot.

Parkinson's patients have fewer accidents if they concentrate only on walking. If they try to do two things as once, they may lose balance or freeze in place. If they want to talk with someone or look at something, they need to stop walking.

These people may be anxious about tripping and falling, so they walk with their heads down and this contributes to falls. Encourage them to fix their eyes on a goal and walk toward it. Also if they hold on to furniture instead of avoiding it, they have more stability. Using a walker can contribute to a stooped posture. Walkers should be avoided if possible.

When turning, the Parkinson's patient should keep his feet spread, take small steps and rock gently from side to side, facing into the turn.

Getting into and out of a chair
When sitting down, the person should back up slowly until his legs touch the seat of the chair. Then he can bend down and sit.

Standing up too quickly can lower blood pressure and cause a person to fall. The following steps are recommended:

  1. Slide forward to the edge of the chair.
  2. Place both feet on the floor, one in front of the other, about 25 cm. apart.
  3. Put weight on the ball of the closest foot.
  4. Sway back and forth to increase momentum.
  5. With a count of "one, two, three, GO!", push down on the arm rests and stand.
  6. Once upright, stand still to regain balance.

Make sure that the chair he uses is heavy enough so that it will not slide when he gets up, or else put his chair against a wall. You can assist by gently pushing on his back as he rises up. A satin pillow on any seat prevents pants and skirts from sticking. Discard plastic covers on chairs since people stick to them and this may cause a fall when they are trying to stand.

Question 4: There are special mobility techniques to use with Parkinson's patients because:
(One or more of the following answers may be correct.)
they have brain impairment.
they always walk outside when it is windy.
their coordination is not normal for walking.
by giving them instructions, they are able to move more easily.

 

 

Getting Out of Bed
Most Parkinson's patients feel stiff and rigid when getting out of bed in the morning. When getting up this procedure is helpful:

  1. Lie on his side at the edge of the bed.
  2. Put legs over the side of the bed.
  3. Using an elbow and a hand from the opposite side, push down on the mattress.
  4. You lift his head as he begins to sit up.

You can also tie a rope on the end of the bed with a knot in the end near the person. He can grab onto the rope and pull himself up. A little heavy table near the bed may also give him support when he begins to stand. You may also put 10 cm. blocks under the legs at the head of the bed. The angle of the bed helps him get to sitting position.

When he goes to bed at night, a Parkinson's patient may wake up all tangled in bed clothing and unable to move. Dressing them in heavier nightclothes - wool stockings, flannel pajamas and covering them with a light blanket keeps them warm with few bulky covers.

If the person falls, he may also freeze so that he cannot think about how to even begin to get up. The best thing to do is to make him comfortable for about 15 minutes. That is about the time it takes for him to regain his strength and help you with getting himself up. If he does fall, when he has rested, bring him to a sitting position. Let him sit up for a few minutes. When you are ready to lift him up be sure that his knees are bent and his feet flat on the floor. If he is small you can stand in front of him, bend your knees and lift him up under his arms. If he is large, use two people to stand on either side and life under his arms. Be sure to tell him what you are going to do and how he can help. Be sure to have him rock forward as you count to three and then pull him up from the floor.

When Freezing Occurs
The inability to move leaves men and women with Parkinson's disease stooped over with their knees bowed and heels off the ground. Trying to move only makes them more unbalanced. To get them moving again, do as follows:

  1. Relax them back onto their heels and raise their toes.
  2. Straighten their posture - they can reach upward to straighten out.
  3. Tap the side of the leg they intend to move first.
  4. Rock gently from side to side or take a step backward.
  5. Walk in place, counting "one, two, three, four."
  6. Start moving forward, heel first.
  7. Keep the feet about 8 inches apart.

Seeing an obstacle in one's path often causes freezing or akinesia. But you can often get the person walking again by placing your foot directly in front of him.

Trying to open a door while walking also causes freezing. Instruct the person the walk up to the door as close as he can, stop, open the door, and then walk through. These are four separate actions.

Question 5: What conditions cause freezing?
(One or more of the following answers may be correct.)
a narrow hallway.
opening a door.
putting a foot in front of the Parkinson's person.
seeing a chair in front of him.

 

 

Constipation
The medications that the Parkinson's patient takes often causes constipation, in fact some people even die from this complication. As the disease progresses, people cannot take in enough water and fiber to stimulate the bowel. The person becomes very uncomfortable and his Parkinson symptoms may increase. Be sure to give these people fluids throughout the day and foods high in fiber such as figs, prunes, and prune juice. There are also stool softeners and fiber supplements to give orally.

Eye Irritation
Although we hardly notice it, we blink our eyes about 8-12 times per minute. The Parkinson's patient blinks his eyes only 2-4 times per minute and thus he looks as if he is constantly staring at you. As a result, he can have conjunctivitis or inflammation of the membrane that lines the eyelids and protects the eye. There is also the opposite problem that some people's eyelids close involuntarily. Usually, they open again by just touching some part of the face.

The blink of an eye is like a windshield wiper that brings tears across the conjunctiva. There are preparations called artificial tears that can be use 4 times a day to help keep the eyes free from infection. In severe cases of eye spasm when the eye opens or closes involuntarily, an ophthalmologist can inject botulism toxin to paralyze the spasm of the muscles.

Seborrhea Dermatitis
Sebborhea is a nuisance problem that leaves an oily coating on the face and scalp. It also causes redness and scaling of the face. It is not clear why Parkinson's patients get this condition. Topical cortisone creams help the skin for short periods of time. Also, look for shampoos that contain selenium sufide, sulfer, or salicylic acid. This condition is incurable and will need to be treated periodically.

Dementia and Depression Between 25-50% of people with Parkinson's also have some Alzhiemer's symptoms. The incidence of dementia is greater in those who develop Parkinson's disease after the age of 60.

Because this disease is so debilitating, about half of them suffer depression. There is some way that the disease process in the brain upsets the neurotransmitters of the brain. It depletes the norepinepherine and serotonin. These are the neurotransmitters that affect mood. So the person's depression is a reaction to his disease and a chemical imbalance. Often, depression is a precursor to Parkinson's. One positive effect of the levodopa drugs is that they alleviate depression.

Question 6: Why should the Parkinson's patient drink fluids during the day?
(Only one of the following answers is correct.)
to prevent constipation.
to help clear his eyes.
to decrease his depression.

 

 

Difficulty Swallowing
When someone cannot swallow properly, he may inadvertently inhale food or liquid into the larynx, down the windpipe, and into the lungs. This can result in aspiration pneumonia. This problem occurs in the late stages of the disease. The tongue loses its strength to adequately push food into the throat and the contractions of the throat to the stomach weaken. Patients frequently say that it feels like there is food caught in their throats. The valve that permits food to enter the stomach may not close properly so that acid juices back up into the esophagus.


How to help the patient eat

To assist the patient, puree the food he eats. You can mix dry baby cereal in juices to make them thick enough so the person does not choke on them. Try giving them 6 small meals during the day instead of three larger ones. These are less tiring for them. Use dishes and spoons that are designed for such persons or buy utensils that a young child would use. Finally, be patient with them. They have a terrible disease and are trying to live the best way they can with many limitations.

If such patients are not eating, a tube maybe inserted directly into the stomach so that liquid nourishment can pushed into it.

Difficulty expelling Secretions
The fading muscle tone that makes swallowing difficult also makes it difficult for these persons to cough up mucous, especially if they have colds. It is hard for these people to control saliva and often they drool saliva from their mouths.

There may be a choking sensation. If you position them on their sides, secretions are less likely to collect in their throats. If the phlegm does not go away, cup your hand and lightly tap on the back. It is easier if you do this while they are sitting up, bent slight forward. This will loosen the secretions and bring them up so that the person can cough them out. The drug amitryptyline dries secretions, so that the person can sleep without difficulty. Give it to him before he goes to sleep and position him on his side. Drugs that contain atropine can also help and they are Probanthine and Artane.

To help the person breath deeper, do breathing exercises with him so that he consciously breaths deeper and exhales more forcefully.

The Future for Parkinson's Disease
As some of you may know, young American actor, Michael Fox, was diagnosed with Parkinson's Disease at the age of 40. He has joined forces with the national organization to continue research on Parkinson's disease. One of the most promising developments for treatment is deep brain stimulation. An electrode similar to a long needle is placed in the brain and connected to an activator unit implanted in the patient's chest. This is much like a cardiac pacemaker. The activator gives off pulsing signals that activate the brain cells. The results have been dramatic for Parkinson's patients and their coordination. The U.S. government has now approved this method of treatment, and studies are being made as to what type of patients would benefit the most from this treatment. The use of micro technology is making changes in how we are able to control disease processes.

The following neurological diseases have problems similar to Parkinson's Disease:

  1. Myesthnia Gravis
  2. Multiple Sclerosis
  3. Muscular Dystrophy

They are easier for the patients to live with, and the progression of these diseases is usually not as dramatic as Parkinson's. Providing care for these patients is similar to caring for those with Parkinson's.

AMYOTROPIC LATERAL SCLEROSIS

This is also a progressive disease similar to Parkinson's. The person may begin to complain of weakness in the extremities, cramping of the muscles, a limping gait. He may have some trouble swallowing and speaking.

With this disease there are 4 classes of motor neuron degeneration:

  1. Progressive muscle atrophy of the spinal cord that helps stabilize the body,
  2. Lateral sclerosis that affects the upper motor neurons of the brain that controls certain parts of the body,
  3. Brain stem paralysis,
  4. The withering of neurons in all three locations.

Of the 4 types, the first is the least aggressive type of disease. The third type is most aggressive because it controls the breathing and swallowing muscles. People with the third type die in one year and the progress of the disease in the other types can be longer.

Here is the story of one man. His name is John. John first felt the effects of ALS in his left foot. He was a businessman who loved to play golf in his free time. After noticing the weakness in his foot, he was playing and suddenly lost his balance, falling to the ground. From his left foot, it progressed up his left side and then down the right side of his body. For the first two years, he was able to do most everything. Then he had to give up driving and helping his wife with the household chores. He couldn't clip his fingernails. In 4 years, he was confined to bed, unable to move any part of his body. He was having trouble breathing and was moved to a rehabilitation hospital. Respiratory therapists helped him to breathe for almost a week, then he became too weak and died.

These illnesses are difficult to understand and difficult to work with on a daily basis. However, people are made in God's image and as we care for them, we care for someone God has seen fit to make. We care for God's creation.

Lecture taken from Complete Bedside Companion, authors McFarlane and Bash, publisher Simon and Schuster, 1998