Update on Guillain-Barré Syndrome

Release Date:  January 1, 2010

Expiration Date: January 31, 2012

FACULTY:

Brittany M. Marshall, PharmD Candidate 2010
Department of Pharmacotherapy
College of Pharmacy
Washington State University
Spokane, Washington

Joshua J. Neumiller, PharmD, CDE, CGP, FASCP
Assistant Professor
Department of Pharmacotherapy
College of Pharmacy
Washington State University/Elder Services
Spokane, Washington

FACULTY DISCLOSURE STATEMENTS:

Ms. Marshall and Dr. Neumiller have no actual or potential conflicts of interest in relation to this activity.

U.S. Pharmacist does not view the existence of relationships as an implication of bias or that the value of the material is decreased. The content of the activity was planned to be balanced, objective, and scientifically rigorous. Occasionally, authors may express opinions that represent their own viewpoint. Conclusions drawn by participants should be derived from objective analysis of scientific data.

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Pharmacists

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Credits: 2.0 hours (0.20 ceu)

Type of Activity: Knowledge

TARGET AUDIENCE:

This accredited activity is targeted to pharmacists and pharmacy technicians. Estimated time to complete this activity is 120 minutes.

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DISCLAIMER:

Participants have an implied responsibility to use the newly acquired information to enhance patient outcomes and their own professional development. The information presented in this activity is not meant to serve as a guideline for patient management. Any procedures, medications, or other courses of diagnosis or treatment discussed or suggested in this activity should not be used by clinicians without evaluation of their patients’ conditions and possible contraindications or dangers in use, review of any applicable manufacturer’s product information, and comparison with recommendations of other authorities.

GOAL:

To discuss the epidemiology, clinical features, and management of Guillain-Barré syndrome (GBS).

OBJECTIVES: 

After completing this activity, participants should be able to:

  1. Explain the epidemiology and clinical features of GBS.*
  2. Discuss the pathophysiology and pathogenesis of GBS.*
  3. Recognize the clinical symptoms and abnormal laboratory measurements indicative of a diagnosis of GBS.
  4. Discuss the management of GBS in terms of immunotherapy, supportive care, and rehabilitation strategies.*

*Also applies to pharmacy technicians.


Guillain-Barré syndrome (GBS) is a peripheral neuropathy characterized by an acute onset with rapidly developing motor weakness.1,2 GBS was first described in 1916 by French neurologists Georges Guillain, Jean-Alexandre Barré, and Andre Stohl upon examination of two soldiers with the condition.3 Guillain, Barré, and Stohl differentiated GBS from the most prevalent cause of acute flaccid paralysis at the time, poliomyelitis, by the finding of elevated cerebrospinal fluid (CSF) protein with a normal cell count.3 The disease is now known to be autoimmune in nature, and is thought to be triggered by a preceding infectious event such as respiratory or gastrointestinal infection.2,4 As illustrated in TABLE 1, GBS can be subdivided into at least four main subtypes of disease, including acute inflammatory demyelinating polyradiculoneuropathy (AIDP); acute motor axonal neuropathy (AMAN); acute motor and sensory axonal neuropathy (AMSAN); and Miller Fisher syndrome (MFS), all of which can differ in terms of worldwide prevalence, clinical presentation, and time to recovery.5,6 This article will discuss GBS in terms of its epidemiology, pathophysiology, presentation, diagnostic features, and clinical management.

EPIDEMIOLOGY

The incidence of GBS is 1.2 to 1.9 cases per 100,000 as indicated by population-based studies conducted in Europe.2,7-11 Men are approximately 1.5 times more likely to be affected than women.8 The incidence of GBS increases with age, with an incidence of 1 per 100,000 in those below the age of 30 years and approximately 4 cases per 100,000 in those over 75 years.8 Some studies have reported a bimodal pattern of incidence by age with peak incidences occurring in young adults and in the elderly.2,7 The most frequent subtype occurring in North America and Europe is AIDP, which accounts for 90% of all GBS cases in these regions.4 In Asia, South America, and Central America, however, the axonal variants of GBS (AMAN and AMSAN) account for 30% to 47% of cases.4 Concerning all subtypes of GBS, approximately twothirds of patients experience an infection within the 6 weeks prior to symptoms, which most commonly involve a respiratory flulike illness or gastroenteritis.12,13 While infectious triggers are thought to be the primary cause of GBS, there are also concerns that certain immunizations may trigger GBS in susceptible individuals.4

PATHOPHYSIOLOGY AND PATHOGENESIS

GBS involves a spectrum of postinfectious autoimmune disorders affecting the peripheral nervous system (PNS), all of which vary slightly in terms of underlying pathophysiology, clinical findings, and course of the disease, as noted in TABLE 1. Each variant results in acute neuropathy, which typically presents as bilateral, symmetrical muscle weakness. The event or conditions that trigger the development of GBS are up for debate; however, it is widely accepted that the underlying cause in a majority of cases can be traced back to a viral or bacterial infection that preceded the onset of GBS. Roughly two-thirds of patients have been found to have recently recovered from an upper respiratory tract or gastrointestinal infection in the 2 to 3 weeks prior to the onset of GBS symptoms.5,14 Several microorganisms, including Campylobacter jejuni, cytomegalovirus (CMV), Haemophilus influenzae, Epstein-Barr virus (EBV), and Mycoplasma pneumoniae, have been cultured from and linked to the infections preceding the onset of GBS.5,14,15

Table 1. Guillain-Barré Syndrome Subtypes
    Axonal Variants Regional Variant
Features AIDP AMAN AMSAN MFS
Frequency
(% of GBS
subtype)
North America
and Europe: 95%
North America and Europe: 5%; China: 60%-80%; Japan, Mexico, and Latin America: 30%-40% Worldwide: 5%  
Associated
infection
Campylobacter jejuni (?) Cytomegalovirus
Epstein-Barr virus
Mycoplasma pneumoniae
C jejuni
Haemophilus influenzae
Cytomegalovirus C jejuni
Target
molecule
Unknown (possibly Schwann cell or myelin sheath) GM1(40%), GM1b, GD1a, and Ga1NAC-GD1a   GQ1b (90%)
Common
symptoms
Bilateral, symmetrical weak- ness starting distally and ascending. Pain in >50% Weakness beginning in muscles of distal extremities. Respiratory muscles usually spared More rapid, severe paralysis. Cranial nerves are involved in most patients Classic triad: areflexia, ataxia, ophthalmoplegia
Autonomic
dysfunction
Can be significant: fluctuating HR and BP, urinary retention Rare; mild if present More frequent than pure motor form May be present; bladder dysfunction
Findings Segmental demyelination, macrophage invasion of myelin. Axons are spared Pure motor axonal conduction block and degeneration Motor and sensory axonal degeneration Cranial nerve inflammation
Nerve
conduction
studies
Slowed conduction
suggestive of demyelination
in 2+ motor nerves.
Absent tendon reflexes
Axonal degeneration without demyelination and/or reversible conduction block. Reduction in muscle action potential without conduction slowing. Hyperreflexia Same as AMAN + sensory involvement Abnormal sensory conduction. Paralysis to the specific region of the body, i.e., cranial nerves. Limbs are normal to mildly abnormal
Recovery Weeks to months at a steady pace. Axonal regeneration can take 6 months Within days (functional nerve conduction failure).Prolonged, poor (from extensive axonal degeneration) Prolonged, poorer recovery than in AMAN Gradual onset, complete recovery over months

AIDP: acute inflammatory demyelinating polyradiculoneuropathy; AMAN: acute motor axonal neuropathy; AMSAN: acute motor and sensory axonal neuropathy; BP: blood pressure; G: ganglioside; GBS: Guilllain-Barré syndrome; HR: heart rate; MFS: Miller Fisher syndrome. Source: Adapted from References 5, 6.

The proposed mechanism for the development of GBS following an otherwise inconspicuous infection is that of molecular mimicry. In other words, the pathogen and target cells of the host appear structurally identical, and the host immune response fails to differentiate between the two. The microorganisms have epitopes on their surface that are structurally similar to molecules (i.e., gangliosides, glycolipids) on the surface of the affected peripheral nerves.5 Gangliosides are a component of the cell membrane in the PNS that function in signal transduction and cell recognition. There are several subsets of gangliosides (i.e., GM, GQ, GD, and GT) and over 50 variants, each distinguished by the number of carbohydrate moieties and sialic acid subunits they contain.4,16 The implicated microorganisms contain structures on their outer membrane that are structural mimics of certain gangliosides.6 During trivial infection, the body recognizes the foreign epitopes on the organism and mounts a T-cell-mediated response followed by a humoral response that results in the production of antibodies directed at the identified epitope. The structural similarity between the pathogen and the nerve cell membrane leads to erroneous recognition and subsequent autoimmune attack of the peripheral nervous tissue (i.e., axons, Schwann cells, nodes of Ranvier) after the infection has cleared.15

C jejuni infection has been found to precede all forms of GBS including MFS, though it is most common in the axonal variants. C jejuni strains contain sialylated lipooligosaccharide subunits that elicit the host antibody response.4,6,15 Cross-reactive C jejuni antibodies tend to target the membrane of the axonal cells (axolemma) and can lead to reversible axonal conduction block or axon degeneration.16 This bacterium is strongly linked to the AMAN variant that presents in China and Japan, with over 60% of these patients showing evidence of C jejuni infection prior to GBS symptoms.6 The C jejuni epitopes also bear resemblance to the GQ1b ganglioside and are therefore also implicated in MFS, where roughly 90% of patients have detectable levels of anti-GQ1b antibodies.4,17

Additional antecedent bacterial infections have been linked to GBS. There is evidence that a particular strain of H influenzae contains an epitope within its cell wall that resembles the gangliosides GM1 and GQ1b. H influenzae infection has been correlated with antibodies to these gangliosides and a diagnosis of AMAN in Japan.6 M pneumoniae contains epitopes that have the potential to generate cross-reactive antibodies to galactocerebroside, a glycolipid present in the PNS myelin.4 Infection with M pneumoniae is associated with 5% of GBS cases and may be implicated in AIDP and AMAN.4

Two viral infections, CMV and EBV, are also thought to play a role in the development of GBS. CMV can cause upper respiratory and flulike illness, and, with a prevalence of 10% to 22%, it is the most common viral trigger of GBS.6 In CMV-associated GBS, antibodies to GM2 are common following CMV infection; however, these antibodies are also common in CMV infected patients who never develop GBS.4 CMV infection often leads to AIDP and is characterized by cranial nerve involvement, respiratory difficulty, sensory deficits, and a delayed early recovery.6,15 Finally, antecedent EBV is linked to AIDP and reportedly results in a milder form of the disease.15

While clear antibody relationships have been documented in the axonal and regional variants of GBS, few patients with AIDP, the most common form of the disease, have been shown to have antibodies to gangliosides.17 There may be an unidentified protein epitope expressed on the Schwann cell that is capable of cross-reacting with antibodies generated following an infection, ultimately leading to myelin dissolution. An alternative hypothesis suggests the damage is primarily cell mediated, whereby activated T lymphocytes recruit activated macrophages to target the Schwann cell surface, releasing toxic mediators that lead to degradation of the cell membrane.4

Concerns regarding the risk of acquiring GBS after vaccination have circulated. The possibility of GBS resulting from vaccination was first raised in the 1976-1977 flu season, in which surveillance reported an increase in the risk of GBS that persisted for 6 to 8 weeks after immunization with the swine flu vaccine, A/New Jersey.3,6 Subsequent analysis has concluded that this risk was only marginally increased, at 1 case of GBS per 1 million vaccinations.3 More recently, a potential increased risk of GBS with those receiving the meningococcal conjugate vaccine Menactra has been reported. Ultimately, the decision to vaccinate a patient with a history of GBS is not clear-cut, and patients should be evaluated on an individual basis. Although the risk of recurrence of GBS is very low after vaccination, the patient and clinician should weigh the benefit of the vaccination against the perceived risk of a second GBS episode.3,6

CLINICAL PRESENTATION

In a typical case of GBS, the patient will notice a combination of pain, numbness, paresthesia, and weakness in the limbs that is symmetrical, bilateral, and progresses proximally.14 The misdiagnosis of patients with GBS is relatively frequent; thus, it is important for clinicians to be familiar with the differential diagnosis of GBS from other forms of acute-onset neuromuscular weakness, as outlined in TABLE 2. The dominant feature in AIDP is weakness that ascends from the legs to the upper limbs, although some patients may not present until all limbs are involved, and around 10% of cases will present with symptoms originating in the upper limbs.3 The progression of GBS is rapid, with weakness reaching clinical nadir in 50% to 90% of patients at 2 to 4 weeks from onset.3,5

Table 2. Differential Diagnosis of Acute-Onset Neuromuscular Weakness

Peripheral Neuropathy

  • Guillain-Barré syndrome
  • Diphtheria
  • Tick paralysis
  • Vitamin B1 deficiency
  • Heavy metal or drug intoxication
  • Acute intermittent porphyria
  • Rabies vaccine induced
  • Vasculitis
  • Critical illness neuropathy

Muscle Disorders

  • Hypokalemia
  • Hypophosphatemia
  • Hypercalcemia
  • Hyermagnesemia
  • Rhabdomyolysis
  • Infection
  • Dermatomyositis

Neuromuscular Junction Disorders

  • Botulism
  • Myasthenia gravis
  • Biologic/industrial toxins

Other

  • Brainstem encephalitis
  • Stroke
  • West Nile virus
  • Poliomyelitis
  • Cord compression
  • Transverse myelitis

Source: References 3, 4, 14, 18.


The muscles innervated by the cranial nerves and the respiratory muscles may also be affected. Weakness of the facial muscles is the most common form of cranial nerve involvement, occurring in about half of cases.3,5 In 25% of cases, respiratory muscle weakness may be severe enough to warrant hospitalization for mechanical ventilation.3,4 Autonomic involvement to varying degrees is common and can include hypertension or hypotension, tachycardia or bradycardia, cardiac arrhythmias, urinary retention, and GI dysmotility.4,5 Recovery is prolonged in most cases, with strength returning over weeks to months. Roughly 5% to 15% of those presenting with a severe case will die.4 Ten to 15% of survivors may still have residual fatigue, weakness, and conduction abnormalities detectable with electrodiagnostic testing, and are more likely to have recurrent episodes of worsening symptoms in the future.3,4

While the axonal and regional variants of GBS display similar symptoms to AIDP, there are some differences, highlighted in TABLE 1. The axonal forms, with minimal autonomic symptoms, tend to develop more rapidly and result in a more severe clinical course, frequently leading to hospitalization and mechanical ventilation.3 AMSAN is distinguished by the presence of combined axonal and sensory symptoms and has a more prolonged recovery period than AMAN.6 The MFS subtype is clinically characterized by a triad of cardinal symptoms: areflexia, paralysis of the extraocular muscles (ophthalmoplegia), and ataxia.6

DIAGNOSIS

The initial diagnosis of GBS is largely based on clinical presentation, with cardinal symptoms including ascending, mostly symmetrical weakness with diminished or absent deep tendon reflexes that has progressed over less than 4 weeks.15 Select diagnostic clues for GBS are outlined in TABLE 3. Progressive paralysis of the limbs and areflexia are considered to be the two essential criteria supporting a diagnosis of GBS.15 Pain, cranial nerve involvement, autonomic disturbances with arrhythmia, and variable blood pressure may also be noted on presentation.14 Lumbar puncture should be performed, as results can aid in the differential diagnosis. Cerebrospinal fluid (CSF) may remain normal in up to 50% of patients early in the disease, but elevated protein levels will be present in more than 90% of patients by the time they reach clinical nadir.5 The CSF white cell count remains unchanged in GBS; therefore, an elevated white count raises the possibility of infection and should steer the clinician to an alternative diagnosis.15 Electrodiagnostic testing is a diagnostic tool utilized when a disease of muscle or of the PNS is suspected, and can be helpful to confirm the diagnosis of GBS and differentiate between subtypes.19 Nerve-conduction studies evaluate nerve function and electrical conduction of the sensory and motor nerves.4,17

Table 3. Diagnostic Clues in Guillain-Barré Syndrome

Supports a Diagnosis of GBS

  • The symptoms have progressed over a few days up to 4 weeks
  • Afebrile at onset
  • Symptoms are relatively symmetrical
  • Autonomic dysfunction present
  • Elevated protein in the CSF with a low white cell count
  • Electrodiagnostic findings are consistent with GBS
  • Bilateral weakness in facial muscles

Doubt a Diagnosis of GBS

  • Bladder or bowel dysfunctr symptoms, or dysfunction that is persistent
  • High white cell count in the CSF (>50 x 106 cells/L)
  • Weakness is markedly asymmetrical
  • Severe pulmonary dysfunction as a primary symptom, before substantial limb weakness

CSF: cerebrospinal fluid; GBS: Guillain-Barré syndrome. Source: Reference 14.


By placing a recording electrode on the skin and stimulating selected nerves, this test can detect signs of conduction block and axonal loss.19 During the needle electrode examination, small needles are placed in the muscle to observe its reactivity during rest and with voluntary stimulation.19 This test may show abnormal spontaneous muscle activity if axonal loss has occurred and is a useful confirmatory test, as early in the disease this test is abnormal in 85% of patients.19 Electrodiagnostic testing should be conducted after initial presentation, preferably more than 3 days from symptom onset, and should be repeated after 1 to 2 weeks regardless of whether the results are initially abnormal or nondiagnostic.4,17,19

MANAGEMENT OF GBS

Most patients with acute GBS require emergency admission and treatment within the hospital setting, where they can be carefully monitored and treated for complications of the disease.17 The following discussion outlines management strategies for patients diagnosed with GBS in terms of immunotherapy, supportive care measures, and rehabilitation.

Immunotherapy

Given that GBS is an autoimmune disorder, therapies utilized in the acute management of the disease involve immunotherapy. The following discusses the use of plasma exchange (also known as plasmapheresis), intravenous immunoglobulin (IVIg), and corticosteroids in the treatment of GBS.

Plasma Exchange: In four clinical trials enrolling 585 patients, plasmapheresis therapy resulted in significant improvements in disability within 4 weeks of treatment, as well as recovery to full strength within 1 year in an increased proportion of patients.20-23 Additionally, results from five pooled studies including 623 participants demonstrated reductions in the percentage of patients requiring ventilation at 4 weeks from 27% to 14% in patients receiving plasma exchange.24 Overall, plasmapheresis was beneficial if administered within 4 weeks of the onset of symptoms, with the benefit of therapy being greater when the treatment was initiated closer to symptom onset.20 Plasma exchange is considered a safe and cost-effective treatment for GBS.3 Plasma exchange is recommended by the American Academy of Neurology for patients with GBS who are considered immobile; however, the use of plasma exchange in patients with mild disease who are ambulant is questionable.25

IVIg: IVIg has become the preferred treatment for severe GBS in recent years, largely due to its relative convenience of administration. Four randomized, controlled trials involving 536 subjects have demonstrated equal efficacy of IVIg and plasma exchange in regard to improved disability at 4 weeks, reduced duration of mechanical ventilation, and prevention of death.3 While plasma exchange has demonstrated efficacy when administered within the first 4 weeks of illness, IVIg use has demonstrated efficacy within the first 2 weeks of illness in clinical trials.4 IVIg is typically administered at a dose of 0.4g/kg/day for 5 days.4 The mechanism of action of IVIg in the treatment of GBS is thought to be multifactorial and is theorized to involve the blockade of Fc receptors, provision of anti-idiotypic antibodies, interference with complement cascade activation, and modulation of T-cell functions.26 It is important to note that trials examining the use of IVIg in combination with either plasma exchange or immunoadsorption have not shown additional benefit over IVIg monotherapy.27 As is the case for plasma exchange, the American Academy of Neurology practice guidelines recommend IVIg for GBS patients with severe disease who are immobile.25

Corticosteroid Therapy: Various studies evaluating the use of corticosteroids for the treatment of GBS have failed to demonstrate clinical improvements in terms of disability after 4 weeks of treatment.28-32 Some of these studies demonstrated less improvement in patients treated with oral steroids when compared to placebo.28-31 These trials additionally failed to demonstrate benefit for other important end points including time to discontinuation of ventilation, time to recovery of unaided walking, death, and documented disability after 1 year.33 One theory concerning the lack of efficacy is that corticosteroid therapy adversely affects the recovery process of GBS by inhibiting macrophage clearance of myelin debris, thus hindering the remyelination process and/or aggravating the damage of denervated muscle fibers.34,35 Supportive Care Supportive care is critical in GBS patients to manage the variety of health issues associated with the condition. Clinical studies in patients with GBS document infection, pulmonary emboli (PE), and cardiac rhythm disturbances as the major causes of death, indicating the importance of supportive care to prevent morbidity as well as mortality in these patients.12,36

Respiratory Function: Respiratory function must be monitored in GBS patients owing to the fact that approximately 25% of patients require mechanical ventilation.3 Respiratory dysfunction seen in GBS patients is due to neuromuscular compromise, with some patients experiencing bulbar dysfunction leading to difficulty clearing respiratory secretions and a subsequent increased risk of aspiration.37,38 Because respiratory dysfunction is common and potentially life threatening, it is important to implement frequent serial respiratory function tests to identify patients experiencing a decline in respiratory status. For those patients who do require mechanical ventilation, further supportive care and rehabilitation are often needed due to complications such as pneumonia, sepsis, and PE.36

Thrombosis Prophylaxis: PE is one of the major causes of death in GBS patients.12 Thrombosis is a risk that must be addressed in GBS patients due to prolonged immobilization, with time to developing deep venous thrombosis (DVT) or PE varying anywhere from 4 to 67 days after disease onset.39,40 Clinical recommendations for patients with GBS are therefore based largely on evidence from other patient populations at risk for thrombosis. Recommendations for patients with GBS include the use of subcutaneous unfractionated or fractionated heparin and support stockings for adult patients who are nonambulant until they are able to walk independently.36 Children have been shown to have a low incidence of DVT, which is likely why this recommendation is limited to adult GBS patients.41

Pain Management: Pain in GBS patients is often localized to the thighs, back, and buttocks, and can be both mechanical and neuropathic in nature.15 In one study prospectively evaluating the incidence and intensity of pain in GBS patients, pain was reported by 89% of subjects and was rated as severe in quality by half of those reporting pain.3 Concerning treatment, analgesics or nonsteroidal anti-inflammatory drugs (NSAIDs) can be used, but often do not provide adequate pain relief for many patients.36 Narcotic analgesics can be used, but given issues in GBS patients with dysautonomia and constipation, these agents must be used cautiously.36

Clinical trial data support the use of gabapentin or carbamazepine in the intensive-care setting during the acute phase of GBS.36 Pregabalin is an additional option for neuropathic pain. According to consensus guidelines, long-term treatment of neuropathic pain can be managed pharmacologically with agents including tricyclic antidepressants, tramadol, gabapentin, carbamazepine, or mexiletine in those refractory to other therapies.36

Blood Pressure and Cardiac Function: Research indicates that between 5% and 61% of GBS patients experience wide fluctuations in blood pressure and can experience cardiac arrhythmias due to autonomic dysfunction.42 Autonomic dysfunction can be so severe as to require cardiac pacemaker placement in patients experiencing severe bradycardia and/or asystole.36 Recommendations call for monitoring of pulse and blood pressure in patients with severe GBS until discontinuation of mechanical ventilation and removal of tracheostomy, or for patients recovering without the need of either intervention.36

Management of Bowel and Bladder Dysfunction: Dysautonomia can lead to bladder and bowel dysfunction in patients with GBS. Constipation is common in patients with GBS, which can be exacerbated by long-term immobilization in the acute phase of GBS and opioid use for pain.36 Estimates state that approximately half of patients with GBS develop adynamic ileus.43 While bladder function studies are limited in GBS, owing to the use of catheterization in most cases, urodynamic studies have documented bladder areflexia and abnormal bladder sensation.44 Monitoring and supportive care should include daily abdominal auscultation and close monitoring of opioid use.36 Erythromycin or neostigmine may be effective pharmacologic interventions for adynamic ileus, but promotility agents should be avoided in patients with dysautonomia.36

Rehabilitation

Owing to the functional disability associated with GBS, a structured multidisciplinary rehabilitation program is widely considered as important as immunotherapy.4 A GBS rehabilitation program utilizes an interdisciplinary team, which can involve physical therapy, occupational therapy, nursing, and social work.45 The goal of a rehabilitation program is to minimize disability and maximize functionality. Rehabilitation programs can include strategies to address mobility issues, respiratory dysfunction, pain, dysautonomia, and fatigue.

Mobility: Physical therapy programs are often needed to regain mobility in patients following acute care for GBS. Mobility programs are typically graduated in nature and include therapy for posture and alignment, maintenance of range of motion, endurance training, strengthening of muscle groups, and flexibility.45

Respiratory Function: A variety of respiratory complications secondary to GBS, including chronic obstructive pulmonary disease, restrictive respiratory disease from pulmonary scarring and pneumonia, trachitis due to chronic intubation, and respiratory muscle insufficiency, can be seen in the rehabilitation setting.45 Techniques such as chest percussion, breathing exercises, and restrictive inspiratory training are often utilized by physical therapists to clear respiratory secretions to aid the breathing process. Weaning protocols are recommended in those patients requiring tracheostomy to prevent excessive fatigue of the respiratory muscles.45

Pain: While a variety of pharmacologic interventions can be used in the management of pain disorders associated with GBS, rehabilitation techniques can also be employed. Occupational therapy and desensitization therapy can be utilized to aid patients to better tolerate activities of daily living that are hindered due to pain.45 In addition to the pharmacologic agents discussed previously, transcutaneous electric nerve stimulation can be used as adjuvant therapy in patients with refractory pain.45

Dysautonomia: Approximately 50% of patients with GBS experience issues with blood pressure control, with postural hypotension being a primary concern in these patients.46 Rehabilitation strategies such as the use of compression stockings, adequate hydration, the use of tilt tables, and postural training can be very beneficial for patients experiencing hypotensive episodes.45 Bladder and/or bowel rehabilitation programs with the goal of maintaining social continence and avoiding complications such as bladder overdistention and urinary tract infections are an important aspect of care.45 Other dysautonomic symptoms, such as impotence, often resolve with time.45

Fatigue: Fatigue, which may result from axonal loss, should be addressed in a rehabilitation program involving both a physical and occupational therapist. There currently exists no evidence to support the use of pharmacologic interventions to treat fatigue in GBS patients; thus, rehabilitation strategies are often the primary treatment. A Cochrane review identified minimal evidence that progressive resistance exercise improves muscle strength in GBS.47 Bicycle exercise training may be beneficial in terms of reduced self-reported fatigue and improved physical fitness, functionality, and quality of life, and could be considered as a component of a structured rehabilitation program.48

CONCLUSION

GBS is a condition associated with both acute and long-term morbidity. Patients with GBS often contend with multiple functional difficulties including, but not limited to, impaired mobility, pain, dysautonomia, respiratory dysfunction, and chronic fatigue. An accurate diagnosis of GBS is critical to ensure appropriate therapy to minimize morbidity and the risk of mortality from this autoimmune disorder. The treatment of GBS is often multidisciplinary in nature and involves immunotherapy, supportive care measures, and long-term rehabilitation programs to regain functionality. Organizations such as the GBS/CIDP Foundation International (www.gbs-cidp. org) can be a great source of information and support for patients, family members and health care providers alike. It is important that pharmacists be aware of the clinical presentation and treatment strategies available for the management of GBS to better serve patients diagnosed with this often devastating condition.

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