Chapter 58

ANS: B Muscles are generally strongest in the morning, and activities involving muscle activity should be scheduled then. Plasmapheresis is not routinely scheduled but is used for myasthenia crisis or for situations in which corticosteroid therapy must be avoided. There is no decrease in sensation with MG. Muscle atrophy does not occur because although there is muscle weakness, they are still used.

13. What should the nurse advise a patient with myasthenia gravis (MG) to do? a. Anticipate the need for weekly plasmapheresis treatments. b. Complete physically demanding activities early in the day. c. Protect the extremities from injury due to poor sensory perception. d. Perform frequent weight-bearing exercise to prevent muscle atrophy.

1. ALS. 2. compromised respiratory function.

1. Progressive muscle weakness and atrophy are the classic signs of 2. Death often results from

1. immunomodulator drugs 2. interferon β-1a (Rebif, Avonex), interferon β-1b, and glatiramer acetate (Copaxone).

1. Treatment for MS begins with use of ______ to modify disease progression and prevent relapse. 2. These drugs include

ANS: B Dalfampridine should not be given to patients with impaired renal function. The other information will not impact whether the dalfampridine should be administered.

10. Which information about a patient with multiple sclerosis indicates that the nurse should consult with the health care provider before giving the prescribed dose of dalfampridine (Ampyra)? a. The patient reports pain with neck flexion. b. The patient has increased serum creatinine. c. The patient walks a mile each day for exercise. d. The patient has the relapsing-remitting form of MS.

ANS: B The Credé method can be used to improve bladder emptying. Decreasing fluid intake will not improve bladder emptying and may increase risk for urinary tract infection and dehydration. The use of incontinence briefs and frequent toileting will not improve bladder emptying.

11. Which action should the nurse plan to take for a patient with multiple sclerosis who has urinary retention caused by a flaccid bladder? a. Encourage decreased evening intake of fluid. b. Teach the patient how to use the Credé method. c. Suggest the use of adult incontinence briefs for nighttime only. d. Assist the patient to the commode every 2 hours during the day.

ANS: B ALS causes progressive muscle weakness. Assisting the patient to perform active ROM will help maintain strength as long as possible. Psychotic manifestations such as agitation and paranoia are not associated with ALS. Cognitive function is not affected by ALS, and the patient's ability to understand procedures will not be impaired. Muscle relaxants will further increase muscle weakness and depress respirations.

15. A patient who has amyotrophic lateral sclerosis (ALS) is hospitalized with pneumonia. Which action should the nurse include in the plan of care? a) Observe for agitation and paranoia. b) Assist with active range of motion (ROM). c) Give muscle relaxants as needed to reduce spasms. d) Use simple words and phrases to explain procedures.

a. Most patients with multiple sclerosis (MS) have remissions and exacerbations of neurologic dysfunction or a relapsing-remitting initial course followed by progression with or without occasional relapses, minor remissions, and plateaus that progressively cause loss of motor, sensory, and cerebellar functions. Intellectual function generally is intact, but patients may have anger, depression, or euphoria. A few people have chronic progressive deterioration, while some may have only occasional and mild symptoms for several years after onset.

20. A 38-year-old woman has newly diagnosed multiple sclerosis (MS) and asks the nurse what is going to happen to her. What is the best response by the nurse? a. "You will have either periods of attacks and remissions or progression of nerve damage over time." b. "You need to plan for a continuous loss of movement, sensory functions, and mental capabilities." c. "You will most likely have a steady course of chronic progressive nerve damage that will change your personality." d. "It is common for people with MS to have an acute attack of weakness and then not to have any other symptoms for years."

c. Specific neurologic dysfunction of MS is caused by destruction of myelin and replacement with glial scar tissue at specific areas in the nervous system. Motor, sensory, cerebellar, and emotional dysfunctions, including paresthesia, patchy blindness, blurred vision, pain radiating along the dermatome of the nerve, ataxia, and severe fatigue, are the most common manifestations of MS. Constipation and bladder dysfunction, short-term memory loss, sexual dysfunction, anger, and depression or euphoria may occur. Excess involuntary movements and tremors are not seen in MS.

21. During assessment of a patient admitted to the hospital with an acute exacerbation of MS, what should the nurse expect to find? a. Tremors, dysphasia, and ptosis b. Bowel and bladder incontinence and loss of memory c. Motor impairment, visual disturbances, and paresthesias d. Excessive involuntary movements, hearing loss, and ataxia

d. There is no specific diagnostic test for MS. A diagnosis is made primarily by history and clinical manifestations. Certain diagnostic tests may be used to help establish a diagnosis of MS. Positive findings on MRI include evidence of at least 2 inflammatory demyelinating lesions in at least 2 different locations within the central nervous system (CNS). Cerebrospinal fluid (CSF) may have increased immunoglobulin G and the presence of oligoclonal banding. Evoked potential responses are often delayed in persons with MS.

22. The nurse should explain to a patient newly diagnosed with MS that the diagnosis is made primarily by a. spinal x-ray findings. b. T-cell analysis of the blood. c. analysis of cerebrospinal fluid. d. history and clinical manifestations.

b. Mitoxantrone cannot be used for more than 2 to 3 years because it is an antineoplastic drug that causes cardiac toxicity, leukemia, and infertility. It is an immunosuppressant given IV monthly when the patient has inadequate responses to other drugs.

23. Mitoxantrone is being considered as treatment for a patient with progressive-relapsing MS. What should the nurse explain is a disadvantage of this drug compared with other drugs used for MS? a. It must be given subcutaneously every day. b. It has a lifetime dose limit because of cardiac toxicity. c. It is a muscle relaxant that increases the risk for drowsiness. d. It is an anticholinergic agent that causes urinary incontinence.

c. The main goals in care of the patient with MS is to keep the patient active and maximally functional, and promote self-care as much as possible to maintain independence. Assistive devices encourage independence while preserving the patient's energy. No care activity should be done by others if the patient can do it for himself or herself. Family involvement in the patient's care and maintaining social interactions are important but not the priority.

24. Priority Decision: A patient with MS is having difficulty with hygienic care due to muscle spasticity and neuromuscular deficits. In providing care for the patient, what is most important for the nurse to do? a. Teach the family members how to care adequately for the patient's needs. b. Encourage the patient to maintain social interactions to prevent social isolation. c. Promote the use of assistive devices so that the patient can take part in self-care activities. d. Perform all activities of daily living (ADLs) for the patient to conserve the patient's energy.

c. The nurse can try to help patients maximize neurologic function and self-care abilities, alleviate physical symptoms, and prevent complications for as long as possible. Many chronic neurologic diseases involve progressive deterioration in physical or mental capabilities and have no cure, with devastating results for patients and families.

34. In providing care for patients with chronic, progressive neurologic disease, what should the nurse identify as the major goal of treatment? a. Meet the patient's personal care needs. b. Return the patient to normal neurologic function. c. Maximize neurologic functioning for as long as possible. d. Prevent the future development of additional chronic diseases.

b. Corticosteroids used in treating acute exacerbations of MS should not be abruptly stopped by the patient because adrenal insufficiency may result; tapering doses should be prescribed and followed. Infection may worsen symptoms and should be avoided. High-fiber diets with vitamin supplements are recommended. Long-term planning for increasing disability is also important.

25. A patient with newly diagnosed MS has been hospitalized for evaluation and initial treatment of the disease. Following discharge teaching, which patient statement indicates that additional instruction is needed? a. "It is important for me to avoid exposure to people with upper respiratory infections." b. "When I begin to feel better, I should stop taking the prednisone to prevent side effects." c. "I plan to use vitamin supplements and a diet high in fiber to help manage my condition." d. "I must plan with my family how we are going to manage my care if I become more incapacitated."

ANS: C Because respiratory insufficiency may be life threatening, it will be most important to monitor respiratory function. The other data also will be assessed but are not as critical.

27. Which assessment should the nurse identify as most important regarding a patient with myasthenia gravis? a) Pupil size b) Grip strength c) Respiratory effort d) Level of consciousness

ANS: B The patient's history and symptoms indicate a possible cholinergic crisis. The health care provider should be notified immediately, and it is likely that atropine will be prescribed. The other actions will be appropriate if the patient is not experiencing a cholinergic crisis.

28. After a thymectomy, a patient with myasthenia gravis receives the usual dose of pyridostigmine (Mestinon). An hour later, the patient reports nausea and severe abdominal cramps. Which action should the nurse take first? a) Auscultate the patient's bowel sounds. b) Notify the patient's health care provider. c) Administer the prescribed PRN antiemetic drug. d) Give the scheduled dose of prednisone (Deltasone).

b. Serum acetylcholine receptor antibodies will confirm a diagnosis of myasthenia gravis (MG). The history and physical revealing weakness is part of the diagnosis, but not the confirmation. Impaired respiratory function is a sign of MG, but not a confirmation of the diagnosis. The EMG will show muscle fatigue with a decreased response.

31. A patient with myasthenia gravis is admitted to the hospital with respiratory insufficiency and severe weakness. What should the nurse recognize will confirm a diagnosis of myasthenia gravis? a. History and physical examination reveal weakness. b. Serum acetylcholine receptor antibodies are present. c. The patient's respiratory function is impaired because of muscle weakness. d. EMG reveals an increased response with repeated stimulation of muscles.

ANS: A Because increased muscle weakness may indicate the onset of a myasthenic crisis, the nurse should assess this patient first. The other patients should be assessed but do not appear to need immediate nursing assessments or actions to prevent life-threatening complications.

32. After change-of-shift report, which patient should the nurse assess first? a) Patient with myasthenia gravis who is reporting increased muscle weakness. b) Patient with a bilateral headache described as "like a band around my head." c) Patient with seizures who is scheduled to receive a dose of phenytoin (Dilantin). d) Patient with Parkinson's disease who has developed cogwheel rigidity of the arms.

c. The patient in myasthenic crisis has severe weakness and fatigue of all skeletal muscles, affecting the patient's ability to breathe, swallow, talk, and move. However, the priority of nursing care is monitoring and maintaining adequate respiratory function.

32. Priority Decision: During care of a patient in myasthenic crisis, what should be the nurse's first priority for the patient? a. Maintaining mobility b. Adequate nutrition c. Maintaining respiratory function d. Maintaining verbal communication

b. Gradual degeneration of motor neurons occurs in ALS, with extreme muscle wasting from lack of stimulation and use. However, cognitive function is not impaired and the patient feels trapped in a dying body. Chorea manifested by writhing, involuntary movements is characteristic of HD. As an autosomal dominant genetic disease, HD has a 50% chance of being passed to each offspring.

33. When providing care for a patient with ALS, what should the nurse recognize as one of the most distressing problems experienced by the patient? a. Painful spasticity of the face and extremities b. Retention of cognitive function with total degeneration of motor function c. Uncontrollable writhing and twisting movements of the face, limbs, and body d. Knowledge that there is a 50% chance the disease has been passed to any offspring

ANS: A Phenytoin can cause gingival hyperplasia, but does not affect bowel sounds, lung sounds, or pupil reaction to light.

5. A patient has been taking phenytoin (Dilantin) for 2 years. Which action should the nurse take when evaluating possible adverse effects of the medication? a. Inspect the oral mucosa. b. Listen to the lung sounds. c. Auscultate the bowel sounds. d. Check pupil reaction to light.

ANS: B Urinary tract problems with incontinence or retention are common symptoms of MS. Chest pain and skin rashes are not symptoms of MS. A decrease in libido is common with MS.

7. What action should the nurse include in completing a health history and physical assessment for a 36-yr-old female patient with possible multiple sclerosis (MS)? a. Assess for the presence of chest pain. b. Inquire about urinary tract problems. c. Inspect the skin for rashes or discoloration. d. Ask the patient about any increase in libido.

ANS: C Symptoms of MS may improve during pregnancy. There is no increased risk for congenital defects in infants born of mothers with MS. Onset of labor is not affected by MS. MS symptoms will not worsen after pregnancy.

8. A woman who has multiple sclerosis (MS) asks the nurse about risks associated with pregnancy. How should the nurse respond? a. "MS symptoms will be worse after the pregnancy." b. "Women with MS frequently have premature labor." c. "Symptoms of MS are likely to improve during pregnancy." d. "MS is associated with an increased risk for congenital defects."

ANS: C Copaxone is administered by self-injection. Oral contraceptives are an appropriate choice for birth control. There is no need to avoid driving or drink large fluid volumes when taking glatiramer.

9. A 33-yr-old patient with multiple sclerosis (MS) is to begin treatment with glatiramer acetate (Copaxone). Which information should the nurse include in patient teaching? a. Recommendation to drink at least 4 L of fluid daily b. Need to avoid driving or operating heavy machinery c. How to draw up and administer injections of the medication d. Use of contraceptive methods other than oral contraceptives

a decreased number of AChR sites at the neuromuscular junction.

Antibodies attack acetylcholine receptors (AChRs) in MG, resulting in

removal of the thymus gland results in improvement in most patients.

Because the presence of the thymus gland appears to enhance the production of AChR antibodies in MG,

Open discussions about their management is essential.

Bladder dysfunction and constipation are major problems with MS.

motor, sensory, cerebellar, and emotional problems.

Common manifestations of MS include

1. chronic, progressive deterioration, 2. remissions and exacerbations.

Disease onset for MS is often gradual, with vague symptoms occurring intermittently over months or years. Some patients have _______ while others have _______.

anticholinesterase drugs, alternate-day corticosteroids, and immunosuppressants.

Drug therapy for MG includes

to prevent major complications of immobility.

During an acute exacerbation, the patient may be immobile and confined to bed. The focus of nursing intervention at this phase is

muscle relaxants.

Many other drugs are used to treat the various symptoms of MS. Spasticity is treated primarily with

Riluzole (Rilutek) and Edaravone (Radicava)

No cure exists for ALS. _______ may slow the progression.

treating the disease process and providing symptomatic relief.

No cure is available for MS. The goal of interprofessional care is

history, manifestations, and the presence of multiple lesions over time on MRI.

No definitive diagnostic test exists for MS. Diagnosis is based on

building general resistance to illness, including avoiding fatigue, extremes of heat and cold, and exposure to infection. Exercise and nutrition are valuable.

Patient teaching should focus on

infectious (viral), immunologic, and genetic factors.

The cause of MS is unknown. MS is related to

Some patients have short-term remissions, others stabilize, and others have severe, progressive involvement.

The course of MG is highly variable.

remains cognitively intact while deteriorating physically.

The illness trajectory for ALS is devastating because the patient

muscle weakness in areas that affect swallowing and breathing.

The major complications of MG result from

moving the eyes and eyelids, chewing, swallowing, speaking, and breathing.

The muscles most often affected by the fluctuating weakness of MG are those used for

Correct answer: d Rationale: Amyotrophic lateral sclerosis (ALS) results in destruction of the motor neurons in the brainstem and spinal cord, causing gradual paralysis. Cognitive function is maintained. Because no cure exists, interprofessional care is palliative and based on symptom relief. Death often occurs within 2 to 5 years after diagnosis.

The nurse is reinforcing teaching with a patient newly diagnosed with amyotrophic lateral sclerosis (ALS). Which statement would be appropriate to include in the teaching? a. "Even though the symptoms you have are severe, most people recover with treatment." b. "ALS results from excess chemicals in the brain, so symptoms can be controlled with medication." c. "You need to consider advance directives now, because you will lose cognitive function as the disease progresses." d. "This is a progressing disease that eventually results in permanent paralysis, though you will not lose any cognitive function."

maintaining adequate ventilation, continuing drug therapy, and watching for side effects of therapy.

The patient with MG who is admitted to the hospital usually has a respiratory tract infection or is in an acute myasthenic crisis. Nursing care is aimed at

(1) evidence of at least 2 inflammatory demyelinating lesions in at least 2 different locations within the CNS, (2) damage or an attack occurring at different times (usually 1 month or more apart), and (3) all other possible diagnoses ruled out.

To be diagnosed with MS, the patient must have

Plasmapheresis and IV immunoglobin G

can yield short-term improvement in symptoms for MG.

Multiple sclerosis (MS)

is a chronic, progressive, degenerative disorder of the central nervous system (CNS) characterized by disseminated demyelination of nerve fibers of the brain and spinal cord.

Amyotrophic lateral sclerosis (ALS)

is a rare progressive neurologic disorder of unknown cause. Motor neurons in the brainstem and spinal cord gradually degenerate, often leading to death within 2 to 5 years.

Myasthenic crisis

is an acute exacerbation of muscle weakness triggered by infection, surgery, emotional distress, exposure to certain drugs, or beginning treatment with corticosteroids.

Myasthenia gravis (MG)

is an autoimmune disease of the neuromuscular junction characterized by fluctuating weakness of certain skeletal muscle groups.