CHAPTER 59 Chronic Neurologic Problems 1439

Nervous System
Objective data should include respiratory rate and depth,
oxygen saturation, arterial blood gas analyses, pulmonary func-
tion tests, and evidence of respiratory distress in patients with
acute myasthenic crisis. Assess muscle strength of all face and
limb muscles, swallowing, speech (volume and clarity), and
cough and gag reflexes.
NURSING DIAGNOSES
Nursing diagnoses for the patient with MG may include, but are
not limited to, the following.
• Ineffective airway clearance related to intercostal muscle
weakness and impaired cough and gag reflex
• Impaired verbal communication related to weakness of
the larynx, lips, mouth, pharynx, and jaw
• Activity intolerance related to muscle weakness and
fatigability
• Disturbed body image related to inability to maintain
usual lifestyle and role responsibilities
ing doses of drugs so that peak action is reached at mealtime
may make eating less difficult. Arrange diversional activities
that require little physical effort and match the patient’s inter-
ests. Help the patient plan activities of daily living to avoid
fatigue. Teaching should focus on the importance of following
the medical regimen, complications of the disease, potential
adverse reactions to specific drugs, and complications of therapy
(crisis conditions) and what to do about them. Explore com-
munity resources such as the Myasthenia Gravis Foundation of
America and MG support groups.
EVALUATION
The expected outcomes are that the patient with MG will
• Maintain optimal muscle function
• Be free from side effects of drugs
• Not experience complications (myasthenic or cholinergic
crises) from the disease
• Maintain a quality of life appropriate to the disease course

PLANNING AMYOTROPHIC LATERAL SCLEROSIS

The overall goals are that the patient with MG will (1) have a
return of normal muscle endurance, (2) manage fatigue, (3) Amyotrophic lateral sclerosis (ALS) is a rare progressive neu-
avoid complications, and (4) maintain a quality of life appropri- rologic disorder characterized by loss of motor neurons. ALS
ate to the disease course. usually leads to death 2 to 6 years after diagnosis, but a few
patients may survive for more than 10 years.26 This disease
NURSING IMPLEMENTATION became known as Lou Gehrig’s disease after the famous baseball
The patient with MG who is admitted to the hospital usually player was stricken with it in 1939. The onset is usually between
has a respiratory tract infection or is in an acute myasthenic 40 and 70 years of age. ALS is more common in men than
crisis. Nursing care is aimed at maintaining adequate ventila- women by a ratio of 2:1. Approximately 5000 people in the
tion, continuing drug therapy, and watching for side effects of United States are diagnosed with ALS every year.26
therapy. Be able to distinguish cholinergic from myasthenic For unknown reasons, in ALS motor neurons in the brain-
crisis (Table 59-21) because the causes and treatment of the two stem and the spinal cord gradually degenerate (see eFig. 59-1
conditions differ greatly. available on the website for this chapter). Dead motor neurons
As with other chronic illnesses, focus care on the neurologic cannot produce or transport signals to muscles. Consequently,
deficits and their impact on daily living. Teach the patient about electrical and chemical messages originating in the brain do not
a balanced diet that can easily be chewed and swallowed. Semi- reach the muscles to activate them.
solid foods may be easier to eat than solids or liquids. Schedul- The typical symptoms of ALS are limb weakness, dysarthria,
and dysphagia. Muscle wasting and fasciculations result from
the denervation of the muscles and lack of stimulation and use.
TABLE 59-21 COMPARISON OF MYASTHENIC Other symptoms include pain, sleep disorders, spasticity, drool-
AND CHOLINERGIC CRISES ing, emotional lability, depression, constipation, and esophageal
reflux.26 Death usually results from respiratory tract infection
Myasthenic Crisis Cholinergic Crisis secondary to compromised respiratory function.
Causes Unfortunately, there is no cure for ALS. Riluzole (Rilutek)
Exacerbation of myasthenia Overdose of anticholinesterase slows the progression of ALS. This drug works to decrease the
following precipitating drugs resulting in increased amount of glutamate (an excitatory neurotransmitter) in the
factors or failure to take ACh at the receptor sites,
brain.
drug as prescribed or remission (spontaneous or
drug dose too low after thymectomy)
The illness trajectory for ALS is devastating because the
patient remains cognitively intact while wasting away. Guide the
Differential Diagnosis patient in the use of moderate-intensity, endurance-type exer-
Improved strength after IV Weakness within 1 hr after cises for the trunk and limbs, since this may help reduce ALS
administration of ingestion of anticholinesterase spasticity. Nursing interventions include (1) facilitating com-
anticholinesterase drugs munication, (2) reducing risk of aspiration, (3) facilitating early
Increased weakness of Increased weakness of skeletal
skeletal muscles muscles manifesting as ptosis,
identification of respiratory insufficiency, (4) decreasing pain
manifesting as ptosis, bulbar signs, dyspnea secondary to muscle weakness, (5) decreasing risk of injury
bulbar signs (e.g., Effects on smooth muscle related to falls, and (6) providing diversional activities such as
difficulty swallowing, include pupillary miosis, reading and companionship,
difficulty articulating salivation, diarrhea, nausea or Support the patient’s cognitive and emotional functions.
words), or dyspnea vomiting, abdominal cramps, Help the patient and family manage the disease process, includ-
increased bronchial secretions,
ing grieving related to the loss of motor function and ultimately
sweating, or lacrimation
death. Discuss with the patient and caregiver issues such as arti-
ACh, Acetylcholine. ficial methods of ventilation and advance directives.