Guillain-Barre Syndrome

Guillain-Barré syndrome (GBS; also known as acute idiopathic demyelinating polyneuropathy) is an acute, rapidly progressing form of polyneuritis that results in a temporary, flaccid paralysis lasting for 4 to 8 weeks. It is the most common cause of acute flaccid paralysis in the United States. Motor, sensory, and autonomic functions may be involved. GBS is actually a collection of syndromes that result from diffuse inflammation or demyelination (or both) of the ascending or descending peripheral nerves that leads to a viral illness and then paralysis.

Although the syndrome is considered to be a medical emergency, over 80% of persons who are affected with GBS recover their functional abilities completely. The remaining individuals have some degree of neurological deficit after recovery from the disease, which results in a chronic disability. Fewer than 5% of patients with GBS die, and usually, death is related to respiratory complications. Other complications include residual numbness, pain, thromboembolic phenomena, pressure sores, and relapse.

Causes ⬆ ⬇

Although the exact cause of GBS is unknown, two-thirds of patients who develop it have had a viral or bacterial infection 1 to 3 weeks before the development of symptoms. It is considered a postinfectious, immune-mediated disease. The most typical site and cause of infections are a lung or intestinal infection caused by Campylobacter jejuni or cytomegalovirus (CMV). Infections with Epstein-Barr virus and Mycoplasma pneumoniae are also associated with GBS. Another 10% of patients have had recent surgical procedures during the 4 weeks before GBS developed. The immune response is directed against the lipopolysaccharide antigens of the bacteria; the antibodies react not only with the bacteria but also with the myelin of the nerves. When the myelin is stripped from the nerves, there is altered propagation of electrical impulses leading to flaccid paralysis. Other diseases that have been linked to the development of GBS are lymphoma, HIV disease, gastroenteritis, Hodgkin disease, and lupus erythematosus. Several cases of GBS have been linked to the novel coronavirus 2019 disease (COVID-19). In some cases, GBS develops after immunization for influenza.

There are two subtypes of GBS. Acute inflammatory demyelinating polyneuropathy (AIDP) is generally preceded by a bacterial or viral infection. Less than half of these patients are seropositive for C jejuni, and symptoms generally resolve with remyelination. Acute motor axonal neuropathy (AMAN) is more prevalent among pediatric age groups, and most of these patients are seropositive for C jejuni. AMAN is identified with rapidly progressive weakness, respiratory failure, and good recovery.

Genetic Considerations ⬆ ⬇

GBS is considered to be an autoimmune disorder, with some reports of familial inheritance. New evidence suggests that polymorphisms in the gene TNFA lead to increased susceptibility to GBS. Rare cases may also be caused by a mutation in the PMP22 gene.

Sex and Life Span Considerations ⬆ ⬇

The incidence of GBS is 1.2 to 3 per 100,000 individuals, and it affects individuals of both sexes and of all ages but is rare in infants. Most commonly, it affects young (15–35 years) and older-aged adults (50–75 years) and men slightly more than women.

Health Disparities and Sexual/Gender Minority Health ⬆ ⬇

There are no known racial and ethnic considerations except that Asian American persons are most likely to have GBS after an infection with C jejuni as compared with other agents. For unknown reasons, active-duty U.S. military personnel have a slightly higher risk for GBS than other groups, particularly in the setting of gastroenteritis. Sexual and gender minority status has no known effect on the risk for GBS.

Global Health Considerations ⬆ ⬇

The underlying cause of GBS varies by country, but the syndrome has been reported in all regions of the world. AIDP occurs most often in Western Europe and North America, whereas people in China, Japan, and Mexico are more likely to have AMAN.

Assessment ⬆ ⬇

ASSESSMENT

History

Determine if the patient has had a recent viral illness or surgical procedure. Often, 2 to 4 weeks after the infection, the patient describes a minor upper respiratory or gastrointestinal febrile illness. Many, but not all, patients complain of finger paresthesia (numbness, prickling, tingling) early in the course of the illness, followed by muscle weakness of the legs. The patient or family generally seeks assistance when bilateral lower limb weakness begins to spread toward the trunk or has progressed to paralysis of the limbs. Urinary incontinence may be a problem initially, followed by difficulty in swallowing and speaking. Impairment of respiratory functions, the most life-threatening effect of GBS, does not occur until the paralysis has affected all of the peripheral areas and the trunk. Often the patient reaches the lowest point of functioning 4 weeks after the start of symptoms.

Physical Examination

The major neurological sign found in GBS is muscle weakness, but symmetrical sensory loss, particularly in the legs and later in the arms, often occurs. Most patients experience pain during their illness and at least half describe severe pain. Although the progression of symptoms is variable, the disease often progresses upward from the legs in 1 to 3 days. To follow the progression of symptoms, test for ascending sensory loss by gently using a pinprick upward from the level of T12 on the vertebral column (level of the iliac crests) to the midscapular point (about T6). Mark the patient's skin with a pen every 4 hours to document changes. Notify the physician immediately if the level reaches T8 or higher, because muscles at that level are needed for breathing. Patients may also have ocular muscle paralysis, loss of position sense, and diminished or absent reflexes.

The function of cranial nerve VII (facial nerve) may be affected, especially in the later stages of the paralysis. To test for facial nerve weakness, inspect the patient's face at rest and during conversation. Have the patient raise both eyebrows, smile, frown, puff out the cheeks, and show both upper and lower teeth. If the facial nerve is involved, the patient may have problems talking, chewing, and swallowing.

Patients often have changes in their vital signs. Rapid or slow heart rates and a labile blood pressure may occur because of the effects on the vagus nerve (cranial nerve X); profuse sweating and facial flushing may also occur. Patients require continuous cardiac monitoring to assess for dysrhythmias. Although respiratory function may be impaired in the later stages of paralysis, the nurse needs to assess the patency of the airway and adequacy of breathing in order to initiate prompt interventions when necessary. Weakness and paralysis usually last 2 to 4 weeks before symptoms begin to improve, and the average time to full recovery is 200 days.

Psychosocial

The patient is alert but paralyzed, and this leads to considerable fear and anxiety both during the initial stages and throughout the course of the disease. As the paralysis ascends, patients' level of anxiety will probably rise; their anxiety level can be reduced with antianxiety agents, and pain control helps manage the condition. The patient and significant others will need a great deal of emotional support to deal with the health crisis.

Diagnostic Highlights

Note that the diagnosis generally is made on the basis of clinical symptoms.

Test	Normal Result	Abnormality With Condition	Explanation
Cerebrospinal fluid (CSF) assay	Protein: 15–60 mg/dL; glucose: 40–70 mg/dL; erythrocytes: 0–5/mcL; leukocytes: 0–5/mcL	Increase in CSF protein without an increase in cell count; lymphocytes might be present but are usually < 20/mcL	High protein levels (as high as 500 mg/dL) are often noted 1–2 wk of illness; peak at 4–6 wk

Other Tests: Most laboratory values are not particularly helpful in the initial diagnosis of GBS. White blood cell counts may show mild leukocytosis; other tests include electromyography, pulmonary function tests, arterial and venous blood gases, and blood cultures. Electromyography and nerve conduction studies may be used to monitor neuromuscular changes.

Primary Nursing Diagnosis ⬆ ⬇

Diagnosis

DiagnosisIneffective airway clearance related to weakness and respiratory muscle paralysis as evidenced by loss of gag reflex and/or dysphagia

Outcomes

OutcomesRespiratory status: Airway patency; Respiratory status: Gas exchange; Respiratory status: Ventilation; Symptom control; Symptom severity; Knowledge: Disease process

Interventions

InterventionsAirway management; Oxygen therapy; Airway suctioning; Airway insertion and stabilization; Cough enhancement; Mechanical ventilation management: Invasive and noninvasive; Positioning; Respiratory monitoring

Planning and Implementation ⬆ ⬇

PLANNING AND IMPLEMENTATION

Collaborative

During the acute phase of the illness, the patient may be in the intensive care unit, particularly to support pulmonary function with oxygenation, endotracheal intubation, and mechanical ventilation. Sequential neurological, cardiopulmonary, and hemodynamic assessments are needed. Some patients have chest physiotherapy ordered, and all require aggressive pulmonary toileting to maintain a patent endotracheal or tracheostomy tube. Most patients need a Foley catheter to manage urinary function. Compression boots limit the risk of thromboembolic complications. Physical and occupational therapy consults should occur early in the illness.

IV immunoglobulin administration may hasten recovery. Plasmapheresis (plasma exchange) may be completed during the early stages (first few days) of the syndrome; research suggests that the procedure may reduce the circulating antibodies and shorten the period of paralysis. Care of the patient who is having plasmapheresis includes monitoring the amount of plasma removed and reinfused and monitoring for any reactions to the fluid replacement. Transfusion reactions should not occur because the patient's own blood is being returned. Complications such as infection and hypertension require pharmacologic management. Other patients may become hypotensive and require fluid boluses or vasopressors.

Pharmacologic Highlights

Medication or Drug Class	Dosage	Description	Rationale
Antihypertensives	Varies with drug	Nitroprusside (Nipride); propranolol (Inderal)	Control hypertensive episodes
Low-molecular-weight heparin (enoxaparin, dalteparin)	Varies with drug	Anticoagulant	Prevents thromboembolism during periods of immobility
Antibiotics	Varies with drug	Chosen on the basis of cultures and sensitivities	Combat urinary or pulmonary infection

Other Drugs: Tricyclic antidepressants are effective to relieve pain associated with paresthesias. Note that muscles are tender (particularly in the trunk, thighs, and shoulders) during range-of-motion and turning procedures. Analgesics may be needed for muscle stiffness, pain, and spasm. Antacids and histamine blockers may be used to reduce the risk of gastrointestinal bleeding. Corticosteroids may be given early in the course of the disease, but their usefulness is unproved, and they are generally considered ineffective by most experts.

Independent

The most important interventions center on maintaining a patent airway and adequate breathing. Teach deep-breathing and coughing techniques. Monitor the patient who is mechanically ventilated for airway obstruction. Use deep endotracheal suction to maintain a patent airway, but monitor the patient carefully for dysrhythmias and a dropping oxygen saturation. Because respiratory complications are seen in 35% to 40% of patients, preparation for intubation or eventually a tracheostomy is appropriate. A viable call system for the patient is vital, and the type will depend on the extent of the paralysis. Cardiac dysrhythmias also occur in GBS, so patients require continuous cardiac monitoring.

Provide passive range-of-motion exercises at least twice a day, turn the patient at least every 2 hours, and use splints and pillow supports to keep the limbs in functional positions. Control the environment to limit the risk of injury from falls. Inspect for integrity of the skin. Frequent eye care is necessary for the individual with cranial nerve VII involvement. Protect the cornea with eye shields (some eye specialists may recommend suturing the eyes closed) and provide eye lubricants. Give mouth care at least every 4 hours.

The patient's psychological state is most important. Use relaxation exercises, include the patient in decision making, and provide frequent explanations of care to decrease the patient's anxiety. Mood swings are to be expected. Encourage the patient to ventilate fears and anger related to the paralysis and the prognosis, and refer the patient for consultation if the patient's emotional needs are not being met. If the patient cannot speak because of endotracheal intubation or paralysis, try to establish communication by having the patient blink once for “yes” and twice for “no.”

Evidence-Based Practice and Health Policy

Sedaghat, Z., & Karimi, N. (2020). Guillain-Barré syndrome associated with COVID-19 infection: A case report. Journal of Clinical Neuroscience, 76, 233–235.

A novel coronavirus (COVID-19) outbreak has led to thousands of infections with many body systems involved. The authors report on one patient who developed acute progressive ascending quadriparesis following diagnosis with COVID-19.
The authors theorized that the virus stimulated inflammatory cells, producing an immune-mediated process that resembled GBS. The authors also noted that it was unclear whether COVID-19 produced the antibodies against molecules in the nervous system that led to GBS or it was the result of the immune response. The authors concluded that it is important to pay attention to the neurological symptoms of COVID-19.

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DRG Information ⬇

Introduction ⬆ ⬇