Guillain-Barre syndrome: management and treatment options for patients with moderate to severe progression.
Guillain-Barre syndrome (GBS) is a syndrome that affects the immune system and attacks the peripheral nervous system. Discussion includes defining GBS as well as its etiology and differential diagnosis. Patients with GBS are not uncommon, and therefore it is important to be educated and to have a more precise understanding. GBS patients need to be treated holistically through emotional and physical support and known effective treatments. Through this article, readers will be able to achieve a thorough understanding of GBS and management options/strategies. Clinical features and manifestation of presenting symptoms will assist in determining initial laboratory studies, imaging, and any other testing that should be performed. Proper and quick diagnosis of GBS will be critical to further optimize treatment options and to decrease the likelihood of further immediate progression. Treatment modalities will be discussed as well as management during the acute hospital course and after discharge from the acute care facility. Discussion will focus on moderate to severe cases and associating treatment plans evaluated from evidence-based practice.
Guillain-Barre syndrome (GBS) is a syndrome that affects the immune system and attacks the peripheral nervous system. The response creates a process that causes peripheral neuropathy and weakness. The incidence of GBS affects about one person out of 100,000. Men and women appear to be affected equally. Acute lower extremity weakness with areflexia or diminished reflexes appears over a rapid amount of time such as within 7 days, which indicates that the process is rapidly progressive (Sharshar, Chevret, & Bourdain, 2003). Research by Hund, Borel, Comblath, Hanley, and McKhann (1993) defines the syndrome as "a demyelinating disorder of the peripheral nervous system," which supports the view that it is mediated by an immune response. It seems as if the preceding infection were within the last month or sooner. Some clinical observations have been linked to the apparent immunologic etiology such as immunoglobulin deposition along the myelin sheaths of the peripheral nerves, antibodies against the peripheral tissue, and the ability to damage Schwann cells (Hund et al., 1993). Hund et al. also states "invasion of macrophages and lymphocytes cause areas of myelin splitting and demyelization." When considering other etiologies of GBS, it does not seem to demonstrate a familial or genetic role, but there seems to be insufficient evidence because of the fact there have not been enough documented cases within immediate families (Geleijns et al., 2004). Some common pathogens that have suggested a link to GBS include Campylobacter jejuni, cytomegalovirus, Mycoplasma pneumoniae, and Epstein-Barr vires (Atkinson, Carr, Maybee, & Haynes, 2006). Serological evidence revealed that C. jejuni was involved in 23% to 45% of cases of GBS in the United States, United Kingdom, Japan, and Netherlands (Kuwabara, 2004). Vaccinations are debatable as to being a cause of GBS, and there has been no sufficient evidence to conclude this thought. According to Atkinson et al. (2006), some of the vaccinations include oral poliovirus, influenza, measles and diphtheria, tetanus, and pertussis. It can be assumed that the vaccinations cause an immune response and are capable of initiating GBS. Immune responses from receiving vaccinations are proven by the sometimes aggravating side effects after the injection, such as fever, site irritation, weakness, and fatigue.
Symptoms can appear fairly quickly after the first manifestations begin to present. Progression of symptoms can take place over hours, days, or longer. According to the National Institutes of Health (2009), many individuals reach their greatest stage of weakness within the first 2 weeks of symptoms, and 90% are at their weakest by the third week of illness. Destruction of the myelin along the peripheral nerves and tissue begin to cause symptoms such as lower extremity weakness, numbness, tingling, and total loss of sensation. As the demyelization gets progressively worse, the symptoms also rapidly worsen. The degree of the peripheral nerve involvement in ascending GBS (most common) involves weakness to the lower extremities that may progress to other nerve areas that affect the trunk, the arms, and the cranial nerves. Respiratory dysfunction may also occur, and the patient may need to be mechanically ventilated to maintain airway and breathing functions. In pure motor GBS, the patient retains sensory function. The type of involvement in descending GBS displays symptoms of ptosis, paralysis of the facial muscles, dysphasia, oculomotor weakness, and cardiac dysrhythmias. The Miller-Fisher syndrome is typically rare and presents with a classic triad of symptoms, which include ataxia, areflexia, and opthalmoplegia. External opthalmoplegia of acute onset is a cardinal presentation. Palmieri (2005) stated that "symptoms of GBS reach a plateau until remyelination occurs and they gradually resolve," unless the patient develops secondary axonal injury and then symptoms and deficits may become permanent. Cardiac dysrhythmias and loss of respiratory function are a possible occurrence because of autonomic dysfunction. Symptoms of respiratory dysfunction, tachycardia, orthostatic hypotension, hypertension, and abnormal vagal responses that may trigger bradycardia are signs of autonomic dysfunction and are caused by lesions on the afferent limb of the baroreflex arch (Sulton, 2001). These lesions disrupt hemodynamic stability, and symptoms must be treated immediately. When autonomic dysfunction occurs, it is optimal to prepare for mechanical ventilation as a precautionary measure. Continued assessment and accurate monitoring of vital signs is also an essential measure.
GBS can be difficult to diagnose but can be diagnosed through inclusion and exclusion criteria, a thorough history and physical examination, and a cerebrospinal fluid (CSF) evaluation. Admission assessments should include onset, location, and duration of current symptoms. It will be crucial to assess any prior infections that may have preceded the onset of the current symptoms. Electrodiagnostic results should be indicative of a demyelization process (Lee & Lee, 2006). Examination of CSF through a lumbar puncture is completed to look at protein content. Typically, CSF shows increased protein content (Hund et al., 1993; see Table 1).
A 33-year-old White woman presented to the emergency department with symptoms of progressive lower extremity weakness for approximately 3 days. The patient stated that symptoms began suddenly with numbness, tingling, and burning that got worse over the past few days. The patient is otherwise healthy with a past medical history significant for transverse myelitis in childhood with a residual of left-sided weakness but still with active functioning. Approximately 1 week before the onset of the present symptoms, the patient complained of an upper respiratory infection that lasted about a week and resolved on its own. The patient denied any other current symptoms. She had not taken any medications at home on a daily basis and did not self-treat the symptoms as described earlier. There were no alleviating factors. On admission to the hospital, the patient was continuously monitored and admitted to an intensive care unit (ICU) with a neurology consult. The patient was immediately prepped for a lumbar puncture to evaluate CSF. On examination of fluid contents, there was an elevation in protein content as suspected.
History and Physical
Assessment skills will be of great importance in the initial stages of evaluation and management. It will be of significant value to determine whether an infectious process preceded the current symptoms, onset and duration of symptoms, and any precipitating factors to assist in ruling out other causes. A detailed physical examination should be performed and documented to assist with the diagnosis of GBS or other neuromuscular-related diseases. Myasthenia gravis may display similar symptoms of GBS and should be ruled out as the presenting diagnosis. According to Palmieri (2005)., "MG is a progressive autoimmune disease that interrupts communication of nerve impulses to voluntary muscles." Neurological symptoms such as diplopia, photophobia, slurred speech, bladder dysfunction, respiratory difficulty, and peripheral neuropathy may occur with myasthenia gravis (Palmieri, 2005). Because of the fact that many of these symptoms may present with GBS as well, myasthenia gravis will need to be ruled out through testing such as electromyography, Tensilon testing, and an assay for Ach-receptor binding antibodies. On physical examination, the patient with GBS may elicit either diminished reflex response to lower extremities or areflexia. Extreme muscle weakness can also present with other occurring symptoms. Cranial nerves should be assessed. Cranial nerve involvement is suggestive of the diagnosis (Atkinson et al., 2006). All cranial nerves may be involved with the exception of cranial nerve VIII. There is not any documented hearing loss or difficulty noted with GBS.
Airway and breathing need to be first approached in management. Because "ventilation depends on skeletal muscle contraction and in polyneuropathic diseases such as Guillain-Barre syndrome, ventilation is compromised by loss of motor innervation to the skeletal muscles of respiration," dysfunction may occur (Hund et al., 1993, p. 436). The patient's airway may need to be maintained through either noninvasive or invasive mechanical ventilation. Basic vital signs such as temperature, respirations, blood pressure, and pulse need to be frequently assessed for accuracy because we know that autonomic dysfunction may occur. In some instances depending on severity, invasive monitoring may be another option in managing the patient. With hypotension or hypertension, an arterial line should be placed.
There are three treatments that are currently being used for the GBS patient. They include the use of supportive care, plasmapheresis exchange, and intravenous immunoglobulin (IVIg; Haldeman & Zulkosky, 2005). Corticosteroid use is debatable. Although the use of corticosteroids seems to be a common practice, current research is leaning to the thought that all patients with GBS may not need to be treated with corticosteroid therapy such as methyl prednisone. IVIg therapy tends to be a preferred method for treatment. IVIg therapy contains antibodies in normal serum that are infused into the patient to counteract the patient's own autoantibodies. Side effects may include headache, chills, fever, skin reaction, and hemolytic anemia (Haldeman & Zulkosky, 2005). The last form of known treatment includes the use of plasmapheresis or exchange therapy. Raphael, Chevret, Hughes, and Annane (2002) states that treatment should be initiated within 7 days of onset of presenting symptoms but was still of benefit when patients were treated up to 30 days after onset. "In moderate GBS, four sessions are superior to two. In severe GBS, six sessions are not significantly better than four" (Raphael et al., 2002). Plasmapheresis involves the use of a machine that works by filtering the plasma and the circulating antibodies out of the patient's blood stream by either centrifugal cell separator or filtration across a semipermeable membrane. The plasma is then replaced with albumin. This process in generally performed more than 10 to 15 days (Sulton, 2001). The exchange sessions can last several hours at a time possibly averaging around 4 hours. The patient may be given a break in between the sessions because the sessions may be somewhat exhausting to the patient. It appears that after the sessions, the patient's symptoms may lessen and areflexia may even disappear because the circulating antibodies are removed. Let us return to the case study. CSF examination showed that increased protein content and symptoms led to the diagnosis of GBS. After being diagnosed, she received a total of five plasmapheresis exchanges. The exchange therapy showed significant improvement on a daily basis when considering the extent of her initial symptoms.
Symptoms of burning, tingling, and numbness to the lower extremities seemed to improve daily. Deep tendon reflexes returned a slight amount everyday. The patient was then moved out of an ICU setting and into rehabilitation for further support, which could last from months to years. She completed approximately 1 week of inpatient rehabilitation with extensive physical and occupational therapy and then was discharged home with family. A supportive family environment is essential and without support would be impossible to go home alone. For many months to years, this patient as well as others will need to continue physical therapy and rehab. This particular patient benefited from a combination of plasmapheresis exchanges and aggressive therapy/nursing care. Nursing care includes more than just hemodynamically monitoring the patient. GBS patients require emotional support as well.
GBS patients tend to experience a significant amount of pain as a result of the neuropathy that is occurring. The pain experience should be personalized because all pain is experienced individually. Symptoms of peripheral neuropathy may be worse for patients with moderate to severe cases. In general, "GBS can be associated with pain in up to 55% to 80% of patients" (McDouall & Tasker, 2004, p. 686). The pain seems to be a musculoskeletal and/or a neuropathic type of pain. Anticonvulsants such as carbamazepine have been used since the 1970s and have been shown to be effective for neuropathic types of pain (McDouall & Tasker, 2004). Gabapentin has also been highly prescribed for neuropathic types of pain. Anticonvulsants are currently being used to treat many types of neuropathy, including diabetic patients. When treating neuropathic pain in GBS patients, McDouall and Tasker (2004) states that "anticonvulsants are at least as effective as opiates." They are a benefit by lessening the sometimes dramatic effects from opiates such as sedation and respiratory depression.
Physical therapy and outpatient rehabilitation are critical factors in prognosis. Many studies have been done and show evidence for improvement of symptoms even up to 18 months after diagnosis. One study completed by El Mhandi, Camels, Camdessanche, Gautheron, and Feasson (2007) showed rapid increase in muscle strength during the first 6 months after onset, with continuing gradual improvement of strength even after 12 months. Intensive therapy after discharge from an acute care facility is essential for improving function. In another study by Hiraga et al. (2005), they used 97 patients and evaluated walking status after treatments. Eight percent of the patients were unable to walk independently after onset, one could walk 28 months after onset and another could walk 57 months after onset. In this study, it was concluded that improvement may even be shown years later and with the possibility of even walking independently (Hiraga et al., 2005). Rehabilitation units are using criteria that is useful for daily functioning and outcomes. Carroll, McDonnell, and Barnes (2003) describe an outcome measure that is used for admission and discharge, which rates 18 activities of daily living called the Functional Independence Measure. Edinburgh states that the Functional Independence Measure "is an instrument that is sufficiently sensitive in detecting disability and change over time during rehabilitation of GBS patients" (Sulton, 2001). A decision will need to be decided between the patient and the practitioner of inpatient versus outpatient therapy in some cases, but in other cases, inpatient therapy will be the preferable option. According to Carroll et al., 40% of all GBS patients will require inpatient therapy.
Deep Vein Thrombosis Prevention
Heparin prophylaxis should be give to prevent deep vein thrombosis. As a result of immobility from neuropathic symptoms, blood clots to the lower extremities may present.
Anxiety and fear will most likely accompany the diagnosis of GBS. Fear of paralysis and functionality will be overwhelming for the patient. Loss of sensation, immobility, and pain may also cause fear. It will be a necessity to assess the patient's level of fear as well as trying to alleviate anxieties if at all possible. A support group will also be crucial, such as family, friends, and healthcare staff. Case management can be available to assist with discharge planning and at-home needs. If overwhelming anxiety or depression persists, antidepressant or anti-anxiety therapy may be initiated. Other issues that will need to be addressed include potential loss of wages from a job, health insurance, and changes that will occur over the next several months to years during recovery.
Patients with GBS may become very frustrated as they may not have immediate mobility even after treatments and therapy. The priority will include stabilizing the patient and prevention of worsening symptoms. After being safely moved out of the ICU, aggressive physical therapy can begin.
When patients are mechanically ventilated, there is always a risk for infection because of an intrusion of the airway with a foreign material (endotracheal tube). Nosocomial or ventilator-acquired pneumonia can be difficult to treat and increase morbidity and overall mortality. Prevention of these types of pneumonia can be done with sterile intubation equipment as well as monitoring for aspiration precautions. The patient's bed should always be elevated 30[degrees] to promote proper drainage and lung expansion while decreasing entry of bacteria.
Many patients with GBS may become paralyzed and as a result will be susceptible to skin breakdown (Sulton, 2001). Frequent assessment of the skin and areas with bony prominences should be checked at least every shift. Areas with redness should be monitored for increased erythema or breakdown. Range of motion exercises as well as frequent position change will help to diminish chances of breakdown along with preventing joint contractures. Family members can be taught to assist with range of motion as well as healthcare staff. Preventing areas of moisture to the skin will also aid in maintaining skin integrity.
"Immobility, decreased gastric motility, dysphagia, and depression can contribute to patient malnutrition" (Sulton, 2001). During the acute phase, enteral feeding may be initiated to prevent aspiration as well as prevention of malnutrition through proper feedings. Feeding tubes should be checked for residuals, patency, and proper position. If intolerance of feeding formula is suspected, the formula should be changed. Patients will need to be evaluated for beginning normal feedings by mouth. Speech therapy can evaluate risk of aspiration and swallowing. Gag reflex should be checked before initiating any meals or medications by mouth. Daily weights need to be checked as well as serum albumin and total protein counts (Sulton, 2001). Feedings should be patient specific for caloric count and proteins according to patient's total body weight and laboratory values. Optimal nutrition is essential for recovery and prognosis because malnutrition can delay healing and promote weakness.
GBS is a progressive neuromuscular syndrome that seems to be caused by an immune-mediated response. It attacks portions of the peripheral nervous system. GBS can have morbidity and mortality rates as high as 4% to 8% even after effective therapy (Carroll et al., 2003). Prompt diagnosis and treatment need to be initiated as symptoms may worsen progressively. Peripheral neuropathy, cranial nerve dysfunction, and respiratory dysfunction remain primary concerns, and patients will need to be monitored continuously in an ICU setting. Because autonomic dysfunction may also occur, patients will need frequent vital signs and telemetry to evaluate for bradycardia, hypotension, or rhythm disturbance. Treatment includes supportive care, plasmapheresis, and IVIg. Moderate to severe cases of GBS may progress faster and need initiation of therapy with initial symptoms. GBS occurs worldwide and without geographical clustering and can occur at any age (Kuwabara, 2004). Because of the incidence of immobility, loss of functionality, and paralysis, GBS should be taken seriously and treated aggressively.
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Questions or comments about this article may be directed to Holly R. Bowyer, MSN ACNP-BC, at firstname.lastname@example.org. She is a certified registered nurse practitioner at the University of Pittsburgh Medical Center, Pittsburgh, PA.
Melissa Glover, MBA BSN RN CEN PHRN, is an administrative supervisor at Physicians Regional Medical Center, Naples, FL.
TABLE 1. Comparison of Myasthenia Gravis and GBS Clinical Features Laboratory Features Myasthenia Fluctuating degree and Serologic tests for gravis variable combination of autoantibodies against weakness in ocular, AChR-Ab or MuSK-Ab bulbar, limb, and respiratory muscles GBS Progressive, fairly Elevated CSF protein symmetric muscle weakness accompanied Normal white blood cell by absent or depressed (albuminocytologic deep tendon reflexes dissociation) Diagnostic Evaluation Differential Diagnosis Myasthenia Clinical presentation Thyroid ophthalmopathy gravis Tensilon test Kearns-Sayre syndrome Ice pack test Brainstem and motor cranial nerve pathology Presence of serum AChR-Ab antibodies Generalized fatigue Presence of serum Motor neuron disease MuSK-Ab antibodies (amyotrophic lateral sclerosis) Repetitive nerve stimulation studies Lambert-Eaton myasthenic syndrome Single-fiber electromyography Botulism Penicillamine-induced myasthenia Congenital myasthenic syndromes GBS Clinical presentation Polyneuropathies CSF analysis Spinal cord disorders Nerve conduction studies Neuromuscular junction disorders Electromyography Muscle disorders Treatment Myasthenia Symptomatic treatment gravis --Anticholinesterase agents Chronic immunomodulating treatment --Glucocorticoids and other immunosuppressive drugs Rapid immunomodulating treatment --Plasmapheresis --IVIG Surgical --Thymectomy GBS Supportive care --Respiratory failure --Autonomic dysfunction --Cardiovascular management --Bowel and bladder care --Pain control --Rehabilitation Disease-modifying treatment --Plasma exchange (plasmapheresis) --IVig Interferon-beta Note. GBS = Guillain-Barre syndrome; AChR-Ab = acetylcholine receptor; MuSK-Ab = muscle-specific tyrosine kinase; IVIg = intravenous immunoglobulin.