Guillain-Barré Syndrome

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Guillain-Barré Syndrome

Anatomic Alterations of the Lungs Associated with Guillain-Barré Syndrome

Guillain-Barré syndrome is a relatively rare autoimmune disorder of the peripheral nervous system in which flaccid paralysis of the skeletal muscles and loss of reflexes develop in a previously healthy patient. In severe cases, paralysis of the diaphragm and ventilatory failure can develop. Clinically, this is a medical emergency. If the ventilatory failure is not properly managed, mucous accumulation with airway obstruction, alveolar consolidation, and atelectasis may develop.

Paralysis of the skeletal muscles develops in response to various pathologic changes in the peripheral nerves. Microscopically, the nerves show demyelination, inflammation, and edema. As the anatomic alterations of the peripheral nerves intensify, the ability of the neurons to transmit impulses to the muscles decreases, and eventually paralysis ensues (see Figure 28-1). Box 28-1 lists other names in the literature for Guillain-Barré syndrome.

The major pathologic or structural changes of the lungs associated with the ventilatory failure that may accompany Guillain-Barré syndrome are as follows:

Etiology and Epidemiology

The annual incidence of Guillain-Barré syndrome is 1 to 2 per 100,000 people in the United States. The mortality rate is 4% to 6%, and the morbidity rate (permanent disabling weakness, imbalance, or sensory loss) is 5% to 10%. Although the condition is uncommon in early childhood, it may occur in all age groups and in either gender. A greater incidence has been noted among people 45 years of age and older, among male subjects, and among Caucasians (the condition is 50% to 60% more common in Caucasians). There is no obvious seasonal clustering of cases.

The precise cause of Guillain-Barré syndrome is not known. It is probably an immune disorder that causes inflammation and deterioration of the patient’s peripheral nervous system. Elevated levels of immunoglobulin M (IgM) antibodies against myelin glycolipid have been found in the serum of patients with Guillain-Barré syndrome. Antibodies that are cell-mediated are thought to be responsible for peripheral nerve demyelination and inflammation. Lymphocytes and macrophages appear to attack and strip off the myelin sheath of the peripheral nerves and leave swelling and fragmentation of the neural axon (see Figure 28-1). It is believed that the myelin sheath covering the peripheral nerves (or the myelin-producing Schwann cell) is the actual target of the immune attack.

The onset of Guillain-Barré syndrome often occurs 1 to 4 weeks after a febrile episode caused by a mild respiratory or gastrointestinal viral or bacterial infection. In about 60% of the cases, Campylobacter jejuni is identified as the cause of the preceding infection. Other precipitating factors include infectious mononucleosis, parainfluenza 2, vaccinia, variola, measles, mumps, hepatitis A and B viruses, Mycoplasma pneumoniae, Salmonella Typhi, and Chlamydia psittaci. Although the significance of the association is controversial, during the nationwide immunization campaign in the United States in 1976, more than 40 million adults were vaccinated with swine influenza vaccine, and more than 500 cases of Guillain-Barré syndrome were reported among the vaccinated individuals, with 25 deaths.

Clinical Presentation

The general clinical history of patients with Guillain-Barré syndrome is (1) symmetric muscle weakness in the distal extremities accompanied by paresthesia or dysesthesias (tingling, burning, shocklike sensations), (2) pain (throbbing, aching, especially in the lower back, buttocks, and leg), and (3) numbness. The muscle paralysis then spreads upward (ascending paralysis) to the arms, trunk, and face. The muscle weakness and paralysis may develop within a single day or over several days. The muscle paralysis generally peaks in about 2 weeks. Deep tendon reflexes are commonly absent. The patient often drools and has difficulty chewing, swallowing, and speaking. The management of oral secretions may be a problem. Respiratory muscle paralysis, followed by acute ventilatory failure, occurs in 10% to 30% of cases.

Although Guillain-Barré syndrome is typically an ascending paralysis—that is, moving from the lower portions of the legs and body upward—muscle paralysis may affect the facial and arm muscles first and then move downward. Although the weakness is commonly symmetric, a single arm or leg may be involved before paralysis spreads. The paralysis also may affect all four limbs simultaneously. Progression of the paralysis may stop at any point. After the paralysis reaches its maximum, it usually remains unchanged for a few days or weeks. Improvement generally begins spontaneously and continues for weeks or, in rare cases, months. About 10% of patients have permanent residual neurological deficits. About 90% of patients make a full recovery, but the recovery time may be as long as 3 years.

If diagnosed early, patients with Guillain-Barré syndrome have an excellent prognosis. The diagnosis typically is based on the patient’s clinical history (e.g., sudden ascending paralysis), cerebrospinal fluid (CSF) findings, and abnormal electromyography (EMG) results. The CSF in patients with Guillain-Barré syndrome shows an elevated protein level (500 mg/dL), without an increased lymphocyte count, called albuminocytologic dissociation. EMG helps to establish the diagnosis and the extent of neurologic involvement. The EMG measures the electrical activity of a muscle in response to nerve stimulation. It also measures the nature and speed of electrical conduction along a nerve.

image OVERVIEW of the Cardiopulmonary Clinical Manifestations Associated with Guillain-Barré Syndrome

The following clinical manifestations result from the pathologic mechanisms caused (or activated) by Atelectasis (see Figure 9-8), Alveolar Consolidation (see Figure 9-9), and Excessive Bronchial Secretions (see Figure 9-12)—the major anatomic alterations of the lungs associated with Guillain-Barré syndrome (when ventilatory failure is not properly managed) (see Figure 29-1).

CLINICAL DATA OBTAINED AT THE PATIENT’S BEDSIDE

The Physical Examination

Autonomic Nervous System Dysfunctions

Autonomic nervous system dysfunction develops in approximately 50% of all cases. The autonomic dysfunction involves the overreaction or underreaction of the sympathetic or parasympathetic nervous system. Clinically, the patient may manifest various cardiac arrhythmias, such as sinus tachycardia (the most common), bradycardia, ventricular tachycardia, atrial flutter, atrial fibrillation, and asystole.

Hypertension and hypotension also may be seen. Although the loss of bowel and bladder sphincter control is uncommon, transient sphincter paralysis may occur during the evolution of symptoms. The autonomic nervous system involvement may be transient or may persist throughout the duration of the disorder.

CLINICAL DATA OBTAINED FROM LABORATORY TESTS AND SPECIAL PROCEDURES

Pulmonary Function Test Findings* (Restrictive Lung Pathology)

FORCED EXPIRATORY FLOW RATE FINDINGS

FVC FEVT FEV1/FVC ratio FEF25%-75%
N or ↓ N or ↑ N or ↓
FEF50% FEF200-1200 PEFR MVV
N or ↓ N or ↓ N or ↓ N or ↓

image

LUNG VOLUME AND CAPACITY FINDINGS

VT IRV ERV RV  
 
VC IC FRC TLC RV/TLC ratio
N

image

NEGATIVE INSPIRATORY FORCE (NIF) ↓


*Progressive worsening of these values is key to anticipating the onset of ventilatory failure.

General Management of Guillain-Barré Syndrome

Guillain-Barré syndrome is a potential medical emergency, and patients must be monitored closely after the diagnosis has been made. The primary treatment should be directed at stabilization of vital signs and supportive care for the patient. Initially, such patients should be managed in an intensive care unit. Frequent measurements of the patient’s vital capacity (VC), negative inspiratory force (NIF), blood pressure, oxygenation saturation, and arterial blood gases should be performed. Mechanical ventilation should be initiated when the clinical data demonstrate impending or acute ventilatory failure.

Good clinical indicators of acute ventilatory failure include the following:

The Bronchopulmonary Hygiene Protocol and Lung Expansion Therapy Protocol should be instituted to prevent or treat mucous accumulation, airway obstruction, alveolar consolidation, and atelectasis.

As in any patient who is paralyzed, the risk of thromboembolism increases. Because of this danger, the patient commonly receives subcutaneously administered heparin, elastic stockings, and passive range-of-motion exercises (every 3 to 4 hours) for all extremities. To prevent skin breakdown, the patient should be turned frequently. A rotary bed or Stryker frame may be required. Blood pressure disturbances and cardiac arrhythmias require immediate attention. For example, nitroprusside (Nipride) or phentolamine (Regitine) are commonly administered during severe hypertensive episodes. Episodes of bradycardia are commonly treated with atropine.

Respiratory Care Treatment Protocols

Oxygen Therapy Protocol

Oxygen therapy is used to treat hypoxemia, decrease the work of breathing, and decrease myocardial work. Because of the hypoxemia that may develop in Guillain-Barré syndrome, supplemental oxygen may be required. However, because of the alveolar consolidation and atelectasis associated with Guillain-Barré syndrome, capillary shunting may be present. Hypoxemia caused by capillary shunting or alveolar hypoventilation is refractory to oxygen therapy (see Oxygen Therapy Protocol, Protocol 9-1).

CASE STUDY

Guillain-Barré Syndrome

Admitting History and Physical Examination

A 48-year-old career U.S. Navy physician visited the hospital base clinic because of the acute onset of severe muscle weakness. He had joined the Navy immediately after medical school. Throughout his time in the service, he had the opportunity to pursue his passion—competitive water-ski jumping. For many years he was the first-place winner at most tournaments, including the nationals held yearly. For almost 25 years, he progressed through the age divisions, always remaining the top seed, always capturing the highest title.

The man was in outstanding physical condition. He was an avid runner and weightlifter, and during the off-season he often traveled to a warm climate to practice his water-ski jumping. He had never smoked and had never been hospitalized. He had an occasional “cold.” About 2 years previously, he had begun to focus all his attention on his 19-year-old son, who was quickly following in his father’s footsteps, having just captured the Men’s Division I championship in collegiate ice hockey.

The man stated that he had felt good until 3 weeks before his admission, at which time he experienced a flulike syndrome for 3 days. About 10 days after returning to work, he noticed a tingling and burning sensation in his feet during his morning patient rounds. By dinner time that same day, the tingling and burning had radiated from his feet to about the level of his knees. Thinking that he was just tired from being on his feet all day, he went to bed early that evening. The next morning, however, his legs were completely numb, although he could still move them. Alarmed, he asked his son to drive him to the clinic. After examining him, his doctor (a personal friend) admitted him for a diagnostic workup and observation.

Over the next 3 days, the laboratory results showed that the patient’s cerebrospinal fluid had an elevated protein concentration with a normal cell count. The electrodiagnostic studies showed a progressive ascending paralysis of the man’s legs and arms. He began to have difficulty eating and swallowing his food. The respiratory care practitioners, who were monitoring his vital capacity, negative inspiratory pressure, pulse oximetry, and arterial blood gas values (ABGs), reported a progressive deterioration in all the values. A diagnosis of Guillain-Barré syndrome was recorded in the patient’s chart.

When the man’s ABGs showed pH 7.29, Paco2 53, image 23, and Pao2 86 mm Hg (on a 2 L/min oxygen nasal cannula), the respiratory therapist called the attending physician and reported his assessment of acute ventilatory failure. The doctor transferred the patient to the intensive care unit (ICU), intubated him, and placed him on a mechanical ventilator. The initial ventilator settings were as follows: intermittent mechanical ventilation (IMV) mode, 12 breaths/min, tidal volume 0.75 L, and Fio2 0.40.

Approximately 15 minutes after the patient was committed to the ventilator, he appeared comfortable. No spontaneous breaths were noted between the 12 intermittent mandatory ventilations per minute. His vital signs were as follows: blood pressure 126/82 and heart rate 68 bpm. He was afebrile. A portable chest x-ray examination revealed that the endotracheal (ET) tube was in a good position and the lungs were adequately aerated. Normal vesicular breath sounds were auscultated over both lung fields. His ABGs were as follows: pH 7.51, Paco2 29, image 22, and Pao2 204. His oxygen saturation measured by pulse oximetry (Spo2) was 98%. On the basis of these clinical data, the following SOAP was documented.

3 Days after Admission

The patient’s cardiopulmonary status had been unremarkable. No improvement was seen in his muscular paralysis. No changes had been made in his ventilator settings over the previous 48 hours. His skin color appeared good. Palpation and percussion of the chest were unremarkable. On auscultation, however, crackles and rhonchi could be heard over both lung fields.

Moderate amounts of thick, whitish, clear secretions were being suctioned from the patient’s endotracheal tube regularly. His vital signs were as follows: blood pressure 124/83, heart rate 74 bpm, and rectal temperature 37.7° C (99.8° F). A recent portable chest x-ray examination revealed no significant pathologic process. His ABGs on an Fio2 of 0.40 were as follows: pH 7.44, Paco2 35, image 24, and Pao2 98. His Spo2 was 97%. On the basis of these clinical data, the following SOAP was documented.

Respiratory Assessment and Plan

N/A

Skin color good; crackles and rhonchi over both lung fields; moderate amount of whitish, clear secretions being suctioned regularly; vital signs: BP 124/83, HR 74, T 37.7° C (99.8° F); CXR: unremarkable; ABGs (Fio2 = 0.4): pH 7.44, Paco2 35, image 24, and Pao2 98; Spo2 97%

A

Begin Bronchopulmonary Hygiene Therapy Protocol (vigorous tracheal suctioning and obtain sputum stain and culture). Begin Lung Expansion Therapy Protocol (10 cm H2O positive end-expiratory pressure [PEEP] to offset any early development of atelectasis). Monitor and reevaluate (4 × per shift).

5 Days after Admission

The patient remained alert and comfortable, except for the ET tube. His muscular paralysis remained unchanged. His skin color appeared good, and no remarkable information was noted during palpation and percussion. Although crackles and rhonchi could still be heard over both lung fields, they were not as intense as they had been 48 hours earlier. A small amount of clear secretions was suctioned from the patient’s ET tube. His vital signs were as follows: blood pressure 118/79, heart rate 68 bpm, and temperature normal. Results of a recent portable chest x-ray examination appeared normal. His ABGs on an Fio2 of 0.40 were as follows: pH 7.42, Paco2 37, image 24, and Pao2 97 mm Hg. His Spo2 was 97%. The sputum culture was unremarkable. On the basis of these clinical data, the following SOAP note was recorded.

Discussion

Guillain-Barré syndrome is a neuromuscular paralysis that ensues after infection with a neurotropic virus. This patient had a classic history of ascending paralysis and paresthesias and the diagnostic finding of elevated protein concentration in the spinal fluid. In this setting, serial measurements of the patient’s vital capacity (VC), negative inspiratory force (NIF), blood pressure, oxygen saturation, and arterial blood gases must be measured and charted. Once respiratory failure supervenes, intubation and respiratory support on a ventilator become necessary. As discussed in this chapter, good clinical indicators of acute ventilatory failure include the following: VC <20 mL/kg, NIF <−25 cm H2O, pH <7.35, and Paco2 >45 mm Hg. As noted by the respiratory practitioner, a progressive deterioration was observed in all of these clinical indicators over a 3-day period.

As shown during the first assessment, when acute ventilatory failure developed, the patient was transferred to the ICU, intubated, and placed on a mechanical ventilator. Shortly after the patient was placed on the ventilator, his arterial blood gas values showed hyperoxia and acute alveolar hyperventilation—both of which were caused by the ventilator settings. The appropriate response was to immediately adjust the ventilator settings by reducing the tidal volume or frequency (or both) and the Fio2. At the time of the assessment, the patient exhibited no evidence of airway obstruction or secretions. Therefore the Bronchial Hygiene Therapy Protocol (Protocol 9-2), was not indicated. Indeed, all that needed to be done at that time was to ensure adequate ventilation and oxygenation on the ventilator.

However, 3 days later, at the time of the second assessment, crackles and rhonchi were heard over all lung fields. Clearly the time had come to initiate the Bronchial Hygiene Therapy Protocol with suctioning and possibly even therapy with mucolytic agents. Because of the risk of atelectasis, the Lung Expansion Therapy Protocol (Protocol 9-3), in the form of PEEP on the ventilator, was indicated. In such a case the sputum should be cultured to see whether any infectious organisms are present.

At the time of the final assessment (2 days later), the clinical indicators for airway secretions had decreased—the rhonchi could no longer be heard over the lung fields, and the small amount of sputum suctioned appeared clear. At that point the down-regulation of the Bronchial Hygiene Therapy Protocol was indicated.

Serial VC or NIF measurements would continue to be made until the patient was ready to be extubated and thereafter for at least several days. Indeed, extubation occurred about 3 weeks after the initiation of mechanical ventilation. The patient recovered without incident and returned to his active lifestyle within a year.

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