Signs and Symptoms
Early signs and symptoms often go unnoticed because they occur gradually. This progressive brain disease is characterized by bradykinesia (slowness of movement) and fine, rhythmic tremors of the hands, arms, legs, jaw, and face. The limbs and trunk become rigid and stiff. The person has difficulty maintaining balance and coordination (ataxia). A shuffling and unsteady gait is common. Postural instability occurs. The effects of the disorder may lead to a state of immobility.
Clients have progressive difficulty with walking, talking, eating, and completing ADL because the disease affects automatic movements. Movement of small muscles that control facial expressions is affected. The person may be unable to blink or smile. As muscles become rigid, the face presents as masklike. The person with parkinsonism often drools. Typical features are seen in Figure 78-4.
In the early years, tremors are regular but typically mild so that they are scarcely noticeable. They may affect only one side, then spread to the other side, which may occur immediately or after a period as long as 15 years. Tremors may start in the fingers, extend to the arms, and finally spread to the entire body. Severe tremors are constant—two to five shakes in a second with the thumb beating against the fingers in a sort of pill-rolling movement. Tremors worsen if the person gets excited, but may cease if he or she moves voluntarily. Tremors disappear during sleep, except in the disease’s final stages. All muscles become rigid, with slightly flexed limbs and slowed movements.
FIGURE 78-4 · Parkinson’s disease signs. (A) "Pill-rolling” tremor (B) Forward stoop and shuffling gait.
Because the disease can affect the neck muscles, the person sits or stands in a stooped position. The arms no longer swing when the person walks, and he or she is unable to shift position quickly to keep balance. The walking gait is characterized as shuffling, a motion that helps to keep the client from falling. If pushed a little, the person loses balance, going faster in the direction of the push.
Although mental changes may accompany this disease, their intensity varies. Common changes include emotional lability (fluctuations) and a slowed thinking process. Many people with Parkinson’s disease become clinically depressed.
Medical and Surgical Treatment
Currently, there is no test to predict Parkinson’s disease. No preventive treatment is known. Although many medications are used, the most effective antiparkinsonism drug found to date is levodopa (L-dopa) (see In Practice: Important Medications 78-3).
Surgery, such as deep brain stimulation, may be performed in carefully selected cases. An electrode is implanted deep in part of the brain that controls movement. Control of the stimulation is via a pulse generator (a pacemaker-like device) placed subcutaneously that attaches wires to the electrodes. Surgery may help clients with advanced Parkinson’s disease or poor responses to medication. It is not effective for all individuals.
Nursing Considerations
Physical therapy promotes activity and enables self-feeding, dressing, and transferring from bed to chair. The person can learn range-of-motion (ROM) exercises for the legs and fingers and ways to maintain balance and to prevent neck muscles from contracting. Exercising does not eliminate tremors but does help prevent rigidity.
Because handling food, chewing, and swallowing are difficult, the person may not eat enough nutritious food. Specific vitamins (except vitamin B) and a high-calorie, high-protein diet are prescribed. Provide teaching for the client and family (see In Practice: Educating the Client 78-2).
While the client is in the healthcare facility, check the consistency of food and teach the client and family about food preparation. They should prepare food that is easy to chew and swallow. Meat should be ground and potatoes mashed; a straw should be provided for liquids. Stress the need to prevent injury, especially burns. The client may be embarrassed by eating difficulties, so encourage him or her to eat. Help the client to make agreeable and safe menu selections.
IN PRACTICE: IMPORTANT MEDICATIONS 78-3
FOR PARKINSON’S DISEASE
• Levodopa (L-dopa, Dopar, Larodopa)—Replenishes missing dopamine, a neurotransmitter found in the brain; helps reduce tremors and rigidity of Parkinson’s syndrome.
• Levodopa plus carabidopa (Sinemet)—The combination drug prevents nausea and promotes the effectiveness of L-dopa.
• Dopamine agonists: pramipexole (Mirapex), ropinirole (Requip)—Mimics effects of dopamine; used to promote consistency of levodopa’s effects.
• MAO B inhibitors: selegiline (Eldepryl), rasagiline (Azilect)— Help to prevent breakdown of dopamine.
• Anticholinergics: benztropine (Cogentin)—Assists in reduction of the tremors of Parkinson’s syndrome.
• Glutamate (NMDA) blocking drugs: amantadine (Symmetrel)— Used alone, may help in early stages of syndrome; used in later stages to reduce dyskinesia.
Nursing Considerations
• L-dopa may cause hemolytic anemia and dyskinesia (involuntary muscle movements).
• The beneficial effects of levodopa tend to diminish or become less consistent as the disease progresses.
• All classifications of Parkinson’s syndrome drugs have significant side effects
Constipation may develop because of lack of physical activity, absence of adequate roughage in the diet, or the effects of various medications. Usually a stool softener, such as docusate sodium (Colace), is given. Encourage fluid intake (at least 4-6 glasses of water and juice each day).
Help the client learn how to use adaptive techniques and devices.Help the family make adaptations to the home and in homemaking.
IN PRACTICE: EDUCATING THE CLIENT 78-2
PARKINSON’S DISEASE
• Be as independent as possible.
• Protect against unnecessary stress and fatigue.
• Use adaptive techniques and devices.
• Protect against injuries.
• Take medications on time. Avoid foods and vitamins that negatively interact with medications.
• Be sure to eat a well-balanced diet with plenty of fluids for optimum functioning.
• Have regular eye examinations to check intraocular pressure.
• Follow pertinent exercises, as prescribed or recommended.
• Use adaptive feeding utensils, as necessary
Families should do a house safety check and eliminate hazards such as throw rugs and highly waxed floors. Rationale: The client has poor balance and can easily fall. Arrangements for bathroom facilities and a bedroom on the first floor may be necessary. Rationale: The client should avoid stair climbing, which can be dangerous and may be impossible. Handrails may need to be installed in bathrooms, stairs, and hallways. Nightlights are also helpful. Rationale: These measures can help the client to be more self-sufficient.
Myasthenia Gravis
Myasthenia gravis (MG) is a chronic autoimmune disorder characterized by episodes of weakness in the voluntary muscles. One type of neurotransmitter (chemical messenger), acetylcholine, cannot transmit messages because antibodies (immune molecules) block, alter, or destroy its receptors at the neuromuscular junction. The thymus gland, which is abnormal in individuals with MG, develops abnormal clusters of lymphoid tissues, including tumors of the thymus gland (thymomas). Generally, these tumors are benign, but malignancies can occur.
Myasthenia gravis most commonly affects women younger than 40 years and men older than 60 years. However, it can occur at any age, including in the neonate and during childhood. MG occurs in all ethnic groups. It is not considered an inherited disorder, and it is not contagious.
Signs and Symptoms
Most often, the eye muscles are affected first. The client looks sleepy and expressionless. Drooping eyelids (ptosis) are common; other facial muscles may be affected, especially those used in chewing, swallowing, coughing, and speaking.
The disease usually evolves gradually. The individual notices that certain muscles feel weak immediately after exercise, but that muscle strength returns with rest. Eventually, the person tires with slight exertion. If respiratory muscles are affected, breathing becomes difficult. If the person is unable to expectorate secretions, pneumonia may develop.
A myasthenic crisis occurs rapidly and is considered an emergency situation. Dysphagia, dysphasia (difficulty in speaking), ptosis, diplopia (double vision), and respiratory distress are the usual manifestations. Maintaining an open (patent) airway can be lifesaving. Pulmonary function studies can be used as predictors of respiratory abilities and indicators of potential myasthenic crisis.
Diagnostic Tests
Because the onset of signs and symptoms is slow, diagnosis is often delayed. In addition, similar signs and symptoms are seen in other disorders.
Antibodies to acetylcholine receptors can be detected by laboratory testing. Most individuals with MG have abnormally high levels of acetylcholine receptor antibodies. Typically, an edrophonium test aids in the diagnosis. Edrophonium chloride (Tensilon) is injected IV. The client with MG will experience a temporary relief of symptoms. Other tests include nerve conduction studies and electromyography. CT and MRI scans can be used to identify thyroid tumors.
IN PRACTICE: IMPORTANT MEDICATIONS 78-4
FOR MYASTHENIA GRAVIS
• neostigmine methylsulfate (Prostigmin)
• pyridostigmine bromide (Mestinon, Regonol)
• Immunosuppressant drugs: prednisone, azathioprine (Imuran), cyclosporine (Neoral), tacrolimus (Prograf)
• Diagnostic drugs: edrophonium chloride (Enlon, Tensilon)
Nursing Considerations
• These medications play a vital role in the client’s ability to swallow and to handle respiratory secretions.
• Medications must be given on time and can be given with food (to minimize side effects).
• Side effects must be reported immediately.
Medical Treatment
Myasthenia gravis can be controlled with several available therapies used to reduce weakness and improve muscle strength. Muscle strength can be increased by promoting neuromuscular transmission. Anticholinesterase agents, such as neostigmine and pyridostigmine (Mestinon), are commonly used. Potent immunosuppressive drugs, such as prednisone, cyclosporine, and azathioprine, can suppress the production of the antibody (see In Practice: Important Medications 78-4).
Nursing Alert For the client with MG, avoid the use of sedatives, tranquilizing drugs, and morphine because these drugs may cause respiratory or cardiac depression.
Plasmapheresis, a procedure in which antibodies are removed from the blood, can be beneficial to the client with MG, as well as with other autoimmune disorders. Additionally, high doses of IV immune globulin may be given to provide the client with a temporary dose of normal antibodies. These procedures may be performed during periods of severe weakness.
Surgical Treatment
In many clients with MG, signs and symptoms are abated with a thymectomy (surgical removal of the thymus gland). A stable, long-term remission is the goal of a thymectomy. Some individuals may be cured by the surgery.
Nursing Considerations
Assure clients that current medications and therapies can greatly improve their muscle strength. Generally, individuals can lead normal or nearly normal lives. The client should also be aware that emotional upsets and infections can intensify the disease and precipitate a crisis. Teaching is an important aspect of care (see In Practice: Educating the Client 78-3).
During acute episodes, tube feedings or total parenteral nutrition (TPN) may be necessary. Suctioning may be needed to remove secretions. Keep an oral suction machine at the client’s bedside; it may be lifesaving in a case of choking or threatened aspiration.
IN PRACTICE :EDUCATING THE CLIENT 78-3
MYASTHENIA GRAVIS
• Wear a MedicAlert tag at all times.
• Use a self-dialing telephone or voice-activated phone. Rationale: These phones are useful in emergencies. A voice-activated phone is necessary if the client cannot dial or hold the receiver.
• Take medications on time. Use an alarm clock as a reminder. Rationale: Medications need to be taken on time to maintain a constant blood level.
• Maintain a regular exercise schedule and conserve energy for essential activities.
• Avoid exposures to temperature extremes. Rationale: They may trigger myasthenic crisis.
• Follow a well-balanced diet to maintain optimum health and strength.
• Avoid exposure to infections.
• Be alert for signs of myasthenic crisis—respiratory distress, muscular weakness, dysphagia, fever and general malaise. Keep a suction device available for emergencies; maintaining an open airway is a priority should crisis occur
Warn the client of the signs of myasthenic crisis and instruct him or her to take precautions regarding medical assistance before the crisis develops. Be prepared to assist with intermittent positive pressure breathing (IPPB) treatments, which are often indicated. In the case of severe respiratory involvement, a tracheostomy may be performed.
Huntington’s Disease
Huntington’s disease (HD), also known as Huntington’s chorea, is a chronic, progressive, hereditary condition in which brain cells in the basal ganglia prematurely die. The disorder involves a combination of physical, intellectual, and emotional symptoms.
A child with a parent who has the HD gene has a 50-50 chance of inheriting the gene. Only children who inherit the gene will develop the disease. Only children who inherit the gene can pass the gene to the next generation. All children who inherit the gene eventually will develop HD. The age of onset varies, although symptoms generally do not appear until the person is older than 30 years.
Signs and Symptoms
The disease progresses at different rates. HD usually starts with abnormal involuntary movements, called chorea, such as fidgeting, jerking, and spasms. Personality changes include irritability, mood swings, depression, loss of judgment, and carelessness. Early intellectual symptoms include difficulty making decisions and learning new things. These symptoms progress to constant writhing and uncontrollable movement, changes in speech and ability to swallow, great weakness, severe personality disorders, and psychosis.
Eventually, the person loses bowel and bladder control and all purposeful movement, and is confined to bed rest. Death usually results from pneumonia or another disorder-related complication associated with immobility.
Diagnostic Tests
A thorough medical and family history is important. A genetic test to search for the HD gene is available. Neurologic tests and laboratory tests can be used to differentiate HD from other nervous system disorders.
Treatment
There is no cure for HD. Symptoms cannot be reversed. Treatment is aimed at symptomatic relief of physical symptoms, such as fatigue, restlessness, or hyperexcitability. Medications can be given for depression.
Nursing Considerations
Initially the client may be able to remain at home until ADL cannot be managed. Home care adaptations can provide a better quality of life. The person with HD can use lamps with a touch base and handrails at the proper height that are strong enough to support full body weight. The person can use an electric razor and toothbrush. To steady the hands and arms, he or she can sit down and rest the elbows on a table when shaving or brushing the teeth.
If the person can swallow thin liquids, he or she can use two straws cut to just above the rim of the glass. Sometimes, a thickening agent such as Thick-It is used to make swallowing easier. Cutting a spot out of a Styrofoam cup for the person’s nose can make drinking easier and safer. Rationale: The person does not have to tip the head back to drink; this measure prevents choking.
Couples at risk may wish to consider genetic counseling before deciding whether to have children.
Amyotrophic Lateral Sclerosis
Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s disease, is a rapidly progressive, fatal neurologic disorder resulting in destruction of motor neurons of the cortex, brain stem, and spinal cord. Voluntary muscle movement gradually degenerates. ALS usually strikes between ages 50 and 70 years and affects more men than women.
Signs and Symptoms
The loss of motor neurons causes muscles to atrophy. Initially, the individual may fall frequently and lose motor control of the hands and arms. The individual experiences weakness, fatigue, and spasticity of the arms. As the disease advances, muscles atrophy, with flaccid quadriplegia. Eventually, the respiratory muscles are involved. The course of the disease is consistent, with no remissions. ALS always progresses to respiratory dysfunction and death, generally within 5 years after onset, although the course of the disease may vary.
Treatment
There is no cure or therapy that will lessen the progress of or reverse the disorder. The first drug shown to prolong survival of ALS clients is riluzole (Rilutek). Additional treatments are palliative only, focusing on support and symptom management.
Nursing Considerations
As with many other neurologic disorders, the nurse can provide physical, emotional, and spiritual support to the client and family. The nurse may provide direction and compassion to the family when discussing subjects such as disease progression and death and dying.
Key Concept The individual with ALS retains intellectual and sensory function throughout the course of the disease.
